1/21. Midface distraction.Since the initial application of distraction osteogenesis to the human mandible by McCarthy, distraction osteogenesis has been used for gradual lengthening of the midface in children with syndromic craniosynostosis, cleft lip and palate, hemifacial microsomia, and midface hypoplasia from other causes. Both external and internal devices are available that permit midface distraction. The background of midface distraction and the development of a Modular Internal Distraction (MID) system that permits widespread use of easily customized, buried distraction devices throughout the craniofacial region are presented. The relative and potential clinical indications for distraction, treatment planning, patient preparation, and possible surgical orthodontic interactions during distraction, as well as a variety of case examples showing the MID system, are discussed.- - - - - - - - - - ranking = 1keywords = mandible (Clic here for more details about this article) |
2/21. Minimally invasive Le Fort III distraction.Recent applications of distraction osteogenesis to the Le Fort III osteotomy in patients with craniofacial dysostosis have proven promising (1-3). Distraction has allowed the midfacial segment to be brought further forward and maintained in position with greater stability when compared with the standard technique of intraoperative advancement. Because no bone grafts or plates must be placed, access incisions are necessary only for performance of the osteotomy. In an effort to minimize the morbidity of the procedure, we have begun performing the Le Fort III osteotomy without the coronal incision. Instead, the nasofrontal junction is approached using the medial aspect of an upper blepharoplasty incision. A lower eyelid and gingivobuccal sulcus incision are also used to complete the osteotomy. This technique has resulted in a shorter operative time and decreased blood loss when compared with the Le Fort III distraction procedure using the standard coronal incision.- - - - - - - - - - ranking = 0.0126596347028keywords = lower (Clic here for more details about this article) |
3/21. Segmental distraction of the midface in a patient with Crouzon syndrome.We treated midface hypoplasia in a 20-year-old woman with Crouzon syndrome using a rigid external distraction device. The patient showed severe exophthalmos and maxillary retrusion, although relatively good occlusion had been achieved by long-term orthodontic procedures. We considered that our patient's particular condition could not be resolved by the usual Le Fort III osteotomy/midface distraction procedure, so we devised a segmental approach. The midface, mobilized by Le Fort III osteotomy, was divided into two segments by Le Fort I osteotomy; each fragment was connected to the rigid external distraction device to be distracted separately. Distraction was begun after 1 day at 1 mm/day. The upper and lower segments were distracted over 17 and 12 days, respectively. The patient's occlusion was fully corrected, and her facial contour was significantly improved. After 3 weeks of consolidation, we removed the distraction device. The clinical course was without complication, and no relapse was observed on the cephalogram or computed tomography scan obtained 1 year after the procedure. Our modified technique was helpful in increasing the usefulness of the external distraction system and in refining the midface distraction procedure.- - - - - - - - - - ranking = 0.0126596347028keywords = lower (Clic here for more details about this article) |
4/21. Crouzonodermoskeletal syndrome.Crouzon craniostenosis [MIM 123500], is identified on the basis of the additional phenotypical manifestations of acanthosis nigricans, vertebral changes and cementomas of the jaws. choanal atresia and hydrocephalus are other features. The molecular defect in CDSS is a point mutation in the FGFR3 gene on chromosome 4p, whereas, the mutation in the Crouzon syndrome is in the FGFR2 gene at 10q25.3-26. An affected girl aged 2 years presented at the UWC dental genetics unit with a prior diagnosis of Crouzon syndrome. choanal atresia had necessitated a permanent tracheostomy, and hydrocephalus was managed by a shunt operation. Clinical examination revealed acanthosis nigricans in the axilliary regions, a diagnosis confirmed by a biopsy of the lesion. Eruption of the primary dentition was delayed with only six out of twenty teeth present. Radiographic examination conducted shortly after birth revealed the presence of several tooth buds in both the maxillae and the mandible. The delayed eruption of the teeth will be of significance in future orthodontic and maxillofacial measures for the improvement of the patient's facial Crouzonodermoskeletal syndrome (CDSS) was separated from the classical appearance. Molecular investigations in the girl and her parents are underway. If the specific mutation in FGFR3 is observed, a positive diagnosis of CDSS will be confirmed and the status of her parents and other family members will be determined. On this basis, appropriate genetic management can be offered to the kindred.- - - - - - - - - - ranking = 1keywords = mandible (Clic here for more details about this article) |
5/21. Subtrochanteric fracture managed by intramedullary nail in a patient with pycnodysostosis.A rare case of a 54-year-old man with pycnodysostosis having subtrochanteric fracture of right femur is being reported. This patient had this fracture after a trivial fall while walking. He had typical facial features, short stature, open fontenella, short stubby hands, high arched furrowed palate and radiological features including open cranial sutures, obtuse angle of mandible, dysplasia of lower end of radius and ulna, notching of lumbar vertebra and generalized skeletal sclerosis. The fracture was treated with an intramedullary nail; the surgery was quite a tedious procedure where we faced per-operative difficulty in reaming. The fracture united in 12 weeks. Extramedullary osteosynthesis might offer a better alternative in such patients as marrow is narrow but healing is normal.- - - - - - - - - - ranking = 1.0126596347028keywords = mandible, lower (Clic here for more details about this article) |
6/21. Multidirectional distraction osteogenesis for Crouzon syndrome: technical note.This report describes multidirectional distraction osteogenesis in a patient with Crouzon syndrome. A RED system and a Hyrax expansion screw were used for lateral expansion of the maxilla. Advancement distraction osteogenesis and a Dyna Form were used for widening distraction osteogenesis of the mandible. The devices were fixed after a quadrangular osteotomy and a bimaxillary midline osteotomy.- - - - - - - - - - ranking = 1keywords = mandible (Clic here for more details about this article) |
7/21. Nager acrofacial dysostosis: an unusual association with both upper and lower eyelid colobomas.Nager acrofacial dysostosis comprises defects of cranio facial region and limbs (mostly upper) with variable associated anomalies. The cranio- facial complex is indistinguishable from the mandibulo facial dysostosis (Treacher Collins syndrome). About 80 cases have been described in the literature. We describe the case of a one-day-old male neonate who presented with the typical features of the disease complex. Although normal life span has been reported, our patient died on the second day due to cardio respiratory failure. We report this case because of its rarity and an unusual associated feature of bilaterally symmetrical upper and lower eyelid colobomas.- - - - - - - - - - ranking = 0.063298173513998keywords = lower (Clic here for more details about this article) |
8/21. New osteotomies for midface advancement in patients with Crouzon syndrome.In some patients with Crouzon's syndrome the typical midface advancement can produce an excessive nasal protrusion which calls for subsequent rhinoplasty. Proposed modification of classical Le Fort III procedure consists in midface osteotomy and external distraction without median orbital walls and nasal component. Limited surgical approach through vestibular incision and split lower eyelids makes the operation quite easy and less extensive. So far, three adult patients were successfully treated according to described modality.- - - - - - - - - - ranking = 0.0126596347028keywords = lower (Clic here for more details about this article) |
9/21. Hallermann-Streiff syndrome: report of case.This syndrome, known also as oculomandibulocephaly, is a symmetric second branchial arch defect resulting in dyscephaly with bird facies and hypoplastic mandibles, among other defects; nearly half of all affected persons show dental anomalies. It should be considered a congenital disorder consisting mainly of developmental anomalies of the skull and facial bones.- - - - - - - - - - ranking = 1keywords = mandible (Clic here for more details about this article) |
10/21. Nager acrofacial dysostosis: evidence for apparent heterogeneity.Nager acrofacial dysostosis is characterized by malar, mandibular, and maxillary hypoplasia, macrostomia, abnormal ears, and radial defects. The lower limbs are usually normal. Two patients were reported with many of these manifestations, in addition to severe facial clefts and limb deficiency. These more severely affected patients were thought to have a severe form of Nager acrofacial dysostosis or a new syndrome. We report on a patient with limb findings of intermediate severity. This patient also had hydrocephalus, micropolygyria, and tetralogy of fallot. This may indicate apparent heterogeneity or a single condition with widely variable expression.- - - - - - - - - - ranking = 0.0126596347028keywords = lower (Clic here for more details about this article) |
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