1/176. Simultaneous maxillary and mandibular distraction osteogenesis with a semiburied device. Distraction osteogenesis is a technique utilizing natural healing mechanisms to generate new bone; it is commonly used to lengthen the hypoplastic mandible. Distraction of the maxilla and mandible as a unit is an obvious extension of the technique. We describe the application of a semiburied distractor to simultaneously lengthen the mandible and maxilla and level a canted occlusal plane in three cases. The indications for bimaxillary distraction are reviewed, including its advantages, disadvantages and limitations. ( info) |
2/176. Congenital facial neuropathy in oculoauriculovertebral dysplasia-hemifacial microsomia (Goldenhar-Gorlin syndrome). Four patients with clinical features of Goldenhar-Gorlin syndrome who showed facial paralysis on clinical examination are presented. The fourth case died following surgery for cleft lip. autopsy revealed hypoplasia of the right facial nerve in its intracranial segment, with small right facial nucleus in the brain stem. Nosological aspects of the Goldenhar-Gorlin syndrome are discussed. Peripheral facial paralysis, as a part of this syndrome, is reviewed in the light of clinical and pathological findings and in its relationship to cardiac anomalies. It is suggested that Goldenhar-Gorlin syndrome is a part of a so-called cardiofacial syndrome. ( info) |
3/176. 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. ( info) |
4/176. 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. ( info) |
| Treacher Collins syndrome presents with cranio-facial deformities of variable expressions and severity, but rarely is it associated with acute airway obstruction of such severity that it necessitates surgical intervention. The authors describe their experience with such a neonate, who presented with acute airway obstruction successfully managed with a tracheostomy. The relevant English literature is reviewed. ( info) |
6/176. Nager syndrome. Problems and possibilities of therapy. The congenital Nager acrofacial dysostosis syndrome is presented, and possibilities and problems in the treatment of these patients are described. A case study of a patient who has been followed from birth to adulthood illustrates the surgical/orthodontic course of treatment and its limits. ( info) |
7/176. Flexor digitorum longus accessorius in the club foot of an infant with Nager syndrome. The case of a male infant is reported who had club foot on the right side and pes adductus on the left side in combination with acrofacial dysostosis; he also demonstrated preaxial anomalies of the upper limbs indicative of Nager syndrome. In addition, an unusual aberrant muscle was discovered during surgical correction of the right club foot. ( info) |
| We report two brothers who presented with mandibulofacial dysostosis, growth retardation, microcephaly, thoracic deformities and conductive hearing loss along with asplenia in one case and aplasia of the gallbladder in the other. The pattern of malformations differs significantly from established syndromes with mandibulofacial dysostosis such as Nager syndrome or Genee-Wiedemann syndrome and also from cerebro-costo-mandibular syndrome. As chromosome analysis revealed normal male karyotypes, we consider this to be a distinct heritable syndrome that may be either autosomal recessive or X-chromosomal recessive. ( info) |
9/176. Imaging the neonatal mandible for accurate distraction osteogenesis. The position of mandibular teeth is difficult to document in the neonatal patient. Panorex images are difficult to obtain in an uncooperative pediatric patient. The new technique presented by the authors uses computed tomographic data to create a curved, reformatted image of the mandible, and generates an image similar to a panorex image. This curved, reformatted mandibular image provides accurate visualization of the mandible and mandibular teeth. This technique allows for precise pin placement and osteotomy in distraction osteogenesis. ( info) |
10/176. Goldenhar's syndrome associated with cardiac malformations. A case of Goldenhar's syndrome associated with cardiac malformations such as single ventricle, atresia of pulmonary artery, and patent ductus arteriosus is described. The association of cardiac malformations with Goldenhar's syndrome is very rare and suggests that it is necessary to perform a careful clinical evaluation in this syndrome whether or not additional malformations may exist in visceral organs. ( info) |