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At least 649 records · Page 36Linked to original sources
PubMed1995

Repeated mandibular lengthening in Treacher Collins syndrome: a case report.

A patient with mandibular hypoplasia associated with Treacher Collins syndrome was treated by bilateral distraction osteogenesis. Since less than optimal length was provided by the first distraction, a second corticotomy was performed in the newly formed bone 6 months after the first distraction. Thus bone gained by distraction osteogenesis was subjected to distraction once again. New bone formation occurred after the second lengthening. This case illustrated that distraction osteogenesis may be applied to the mandible at the site of previous distraction.

Bone Lengthening↗
PubMed1993

Natural coral skeleton used as onlay graft for contour augmentation of the face. A preliminary report.

The skeletons of two different genera of Madreporaria were used as onlay graft material for contour augmentation of the face. With coral blocks or granules in a subperiosteal fashion, patients with hemifacial microsomia, maxillary hypoplasia, microgenia, and Treacher Collins' syndrome have been adequately treated. Sixteen augmentations were performed in 14 patients, followed up for 6-34 months. One graft had to be removed because its inadequate fixation resulted in mobility. Delayed healing was observed in another case for the same reason. Coral skeleton is a cheap, natural biomaterial with excellent biocompatibility. It can easily be prepared and shaped, and it maintained its form over the period observed.

Adolescent↗
PubMed2006

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.

Base Sequence↗
PubMed1995

The use of the temporalis muscle flap in facial and craniofacial reconstructive surgery. A review of 182 cases.

The authors report on their 16-year experience of reconstruction with the temporalis myofascial flap in 182 cases. All aspects of reconstructive cranio-maxillofacial surgery are covered: trauma, deformities, tumours, TMJ ankylosis, facial paralysis. The temporalis myofascial flap was used both as a single and as a composite flap with cranial bone, coronoid process or skin island. Major complications were not observed. On the basis of their experience, the authors confirm the reliability, versatility and reproducibility of the use of this flap. This is due both to its rich blood supply and to its proximity to the reconstruction site. It is suggested that the use of the temporalis muscle flap should be taken into consideration before deciding on more extensive reconstructive procedures.

Adolescent↗
PubMed1997

Submerged intraoral device for mandibular lengthening.

The authors report a new technique for mandibular distraction. Lengthening of the mandible by gradual intraoral distraction was obtained in nine young patients. An intraoral device was used in order to avoid external scars. Seven patients had hemifacial microsomia, one patient had a ramus hypoplasia after TMJ ankylosis and one patient had the Treacher-Collins syndrome. The amount of mandibular lengthening ranged from 12 to 28 mm depending on the duration of expansion. Retention after expansion, to allow ossification to take place, lasted for 3 weeks on average. The follow-up period ranged from a minimum of 5 months to a maximum of 44 months.

Ankylosis↗
PubMed1999

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.

Biomechanical Phenomena↗
PubMed1998

Treacher-Collins syndrome and co-existing dermatomyositis.

BACKGROUND: Treacher-Collins syndrome, an autosomal dominantly inherited malformation of structures derived from the first and second branchial arch, has an incidence of 1:10,000 newborns. The prevalence of dermatomyositis at less than 24 years of age has been estimated at 1 per 100,000. The occurrence of both Treacher-Collins syndrome and dermatomyositis combined in the same patient should occur once in every 1,000,000,000 subjects. METHODS: We report a patient with Treacher-Collins syndrome who developed dermatomyositis at the age of 5 years. RESULTS: No other patient with both Treacher-Collins syndrome and an autoimmune disease has been reported. The thymus originates from the third branchial pouch and is unaffected by the syndrome. In Treacher-Collins syndrome the affected gene has been mapped to the fifth chromosome, while dermatomyositis is related to HLA B8 and DR3, coded on the sixth chromosome. No immunologic alteration has been described in patients with Treacher-Collins syndrome. CONCLUSION: This is the first report of a patient with Treacher-Collins syndrome and dermatomyositis. There is no genetic or physiopathologic explanation for the concurrence of both conditions.

Anti-Inflammatory Agents↗
PubMed1971

Choannal atresia.

Explore the source record for details and available documents.

Asphyxia↗
PubMed1977

Goldenhar syndrome: a case report with review of literature.

A case of Goldenhar Syndrome, in an adult male, with the typical triad of auricular appendages, epibulbar dermoid and vertebral anomalies is presented. The relevent literature is review. The differential diagnosis of this Syndrome from a few similar syndromes is stressed.

Abnormalities, Multiple↗