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At least 397 records · Page 22Linked to original sources
PubMed1998

Oro-palatal dysplasia Bettex-Graf--a new syndrome.

Four cases of an until now undescribed syndrome have been observed in Berne in the last 40 years. All four cases are members of the same family and have occurred in three consecutive generations. They present with a U-shaped palatal cleft, microstomia, hypoplasia of the mandibula and a partial anodontia. An autosomal dominant heredity was demonstrated. Karyograms have been made in three of the patients and in all patients showed an anomaly in the form of a "fragile site" in one chromosome (16 fra 16 [q22]). Surgical and orthopedic treatments were difficult.

Abnormalities, Multiple↗
PubMed2002

Tooth agenesis and craniofacial morphology in an orthodontic population.

Previous reports have produced conflicting conclusions regarding the relationship among tooth agenesis, the congenital absence of teeth, and variations in craniofacial morphology. Traditional cephalometric assessment and computer-assisted morphometric analysis of jaw size were used to reexamine this relationship in a white orthodontic population. A positive diagnosis of tooth agenesis (including third molar determination) was made in 89 subjects of mixed age and sex who previously had orthodontic treatment but who were not determined to have a recognized syndrome or a craniofacial anomaly. Cephalograms for these subjects were compared with control data from subjects reported in Bolton Standards of Dentofacial Developmental Growth. Missing teeth and their locations were correlated with changes in selected linear and angular measurements of the Bolton cephalometric analysis. Area measurements of the jaw size of the subjects grouped by age were compared with Bolton cephalometric templates. Relatively little correlation was found between missing teeth and changes in cephalometric measurements. Where identified, a decreased maxillary jaw size and maxillary tooth agenesis were generally associated. Two-dimensional area measurements showed that most age groups had significant decreases in maxillary jaw size associated with tooth agenesis. Relatively few groups had significant changes in mandibular size associated with tooth agenesis.

Adolescent↗
PubMed2003

Facemask therapy with rigid anchorage in a patient with maxillary hypoplasia and severe oligodontia.

In this report, we describe the treatment of a 10-year-old girl with a Class III skeletal relationship with maxillary hypoplasia and severe oligodontia. The maxillary arch was in a complete crossbite relationship with the mandibular arch. The treatment plan called for displacing the maxillary complex anteriorly with a facemask. Because of the lack of available teeth, a rigid anchorage implant was used in combination with the remaining teeth to provide anchorage. A titanium lag screw was placed in the maxillary alveolus. Three weeks later, 800 g of orthodontic force was applied. A significant anterior displacement of the nasomaxillary complex was achieved with the facemask. At the end of treatment, a temporary removable partial denture was placed.

Anodontia↗
PubMed2003

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.

Cephalometry↗
PubMed1998

Sizes of dental arches in young adult patients with Pierre Robin sequence and isolated cleft palate.

The dental arch dimensions of 29 patients with Pierre Robin sequence (16.4-25.4 years of age) and of 31 patients with isolated cleft palate (16.9-19.9 years of age) were examined. All patients in the isolated cleft palate group and 21 patients in the Pierre Robin sequence group had had the Cronin modification V-Y pushback technique primary surgery performed. The Veau-Wardill-Kilner modification had been used in eight Pierre Robin sequence patients for primary surgery. With regard to patients whose height growth was nearly finished, both the upper and lower dental arch sizes were smaller in patients with Pierre Robin sequence than those in patients with isolated cleft palate. On comparison of the arch sizes, sex differences were more pronounced in the isolated cleft palate group. The high percentage of missing teeth and surgery was found to explain some of the small sizes of the dental arches in the Pierre Robin sequence group.

Adolescent↗
PubMed2002

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.

Adolescent↗
PubMed2005

Long-term skeletal effects of mandibular symphyseal distraction osteogenesis. An implant study.

The purpose of this study was to investigate the long-term skeletal effects of mandibular symphyseal distraction osteogenesis (MSDO) with a tooth- and bone-borne distraction device, analysed using the metallic implant method. The study sample comprised 20 patients between 15.8 and 25 years of age, with a mean age of 20.01 +/- 2.25 years at the start of treatment. In 12 subjects, titanium implants were inserted in the mandible to analyse mandibular skeletal changes in the short and long term. A custom-made intraoral, tooth- and bone-borne distractor was designed and used. After a latency period of 7 days, the distractor was activated twice daily, by a total amount of 1 mm. Postero-anterior (PA) cephalograms were obtained at the start of distraction and at the end of consolidation (94.95 +/- 5.79 days after surgery) and follow-up periods (21.5 +/- 4.6 months after consolidation). The data were analysed statistically using paired t-tests. The mean amount of screw activation was 8.10 +/- 1.68 mm. The inter-symphyseal and inter-molar implant distances and the bimolar width significantly increased during the consolidation period (P < 0.001) and were maintained at the end of the follow-up. On the other hand, the bicondylar width was markedly decreased (P < 0.05), while no significant skeletal changes were observed in bigonion and biantigonion widths, inter-ramal implant distance, or inter-ramal and implant angles at the end of the consolidation period. The long-term findings of this study indicate that MSDO provides an efficient and stable non-extraction treatment alternative, mainly by increasing the anterior mandibular skeletal and dental arches.

Adolescent↗
PubMed1983

Teratogenic effects of biotin deficiency in mice.

Teratogenic effects of maternal biotin deficiency produced by a diet containing egg white were studied in mice. More than 90% of the fetuses from biotin-deficient females showed external or skeletal congenital abnormalities. The predominant malformations were micrognathia (91%), cleft palate (82%) and micromelia (41%). Simultaneous supplementation of biotin at the level of 10 mg/kg in the diet thoroughly prevented these malformations. Thus congenital malformations observed in this study were apparently due to the maternal dietary deficiency of biotin during pregnancy. Even though so many fetuses were malformed and many were also stunted, their dams did not exhibit any overt signs of biotin deficiency such as alopecia, dermatitis or nervous irritability.

Abnormalities, Drug-Induced↗
PubMed2004

"Back to sleep" and unexplained death in infants.

OBJECTIVE: Investigation of body position in infants with sudden and apparently unexplained death. Determination of the upper airway space of the infants in different positions by computed tomography (CT) scan. Comparison of the CT scan, the body position at death scene, and the autopsy results. DESIGN: Prospective investigation on all infants referred to a specialized center investigating abrupt and apparently unexplained death of infants. SUBJECTS: Full-term infants with sudden and clinically unexplained death. Four extra infants studied at different postmortem times to verify absence of change in measurement in postmortem CT scan over time. DATA COLLECTED: Position of infant when found dead. CT scan of upper airway in 3 positions (prone face down, prone head rotated, supine nose up), presence or absence of upper airway obstruction, level and length of the obstruction, presence or absence of cause of death, and presence or absence of small maxillomandibular complex at autopsy. RESULTS: Twenty-seven children had unexpected crib deaths (17 of them determined to be sudden infant death syndrome at autopsy). Fourteen children were found dead in the prone position; for 8 of them, this was their normal sleeping position. Airway occlusion behind the base of tongue was seen on CT scan in 24 of the 27 infants (89%) when placed prone head down; in 13 (48%) when placed prone with the head rotated to the side, and in 5 (18%) infants in the supine position. Four infants had mild facial dysplasia and had been found dead in the supine position; in 3 of them, sudden infant death syndrome was found to be the cause of death. The stability of CT scan findings over time was demonstrated after death in 3 different body positions. CONCLUSION: Supine sleeping position may not be necessarily protective when small jaws are present, and sleeping position may also be a factor in abrupt deaths in infants even if "explained by autopsy."

Airway Obstruction↗
PubMed1993

A new osteotomy for genioplasty--stepped osteotomy: preliminary report.

A new osteotomy for genioplasty, stepped osteotomy, is described. This osteotomy is a combination of horizontal and sagittal osteotomy of the mandibular symphysis. It consists of horizontal osteotomy at a distance of 3 to 4 mm below the apexes of the anterior teeth, connecting at right angles with the conventional horizontal osteotomy below the mental foramen, making a step-fashioned osteotomy of the chin. This procedure is particularly advantageous in simultaneous elongation and advancement genioplasty because the central portion between the apexes of the anterior teeth and the conventional horizontal osteotomy line is available as augmentation and the bony contact of the osteotomized mandible can be retained in elongating the chin. The characteristics of this procedure are described and typical patients are shown.

Adolescent↗
PubMed1995

Skeletal distraction of the hypoplastic mandible.

Six patients underwent percutaneous pin placement, 17 intraoral corticotomies, and application of an Orthofix lengthening device to facilitate complex multiplanar distraction as mandated by the patient's specific deformity. The patients underwent gradual bony and soft-tissue distraction at the rate of .25 mm four times a day. Average distraction was 18.3 +/- 5.5 mm (mean +/- SD). After distraction, a 2-day period of stabilization for each 1 mm of lengthening was used (36.4 +/- 10.7 days) (M +/- SD). All the patients had marked improvement in their occlusion postoperatively and significant amelioration of their preoperative respiratory and feeding difficulties. The patients all exhibited dramatic aesthetic improvement. Intraoral corticotomies performed in conjunction with gradual skeletal distraction appears to offer significant advantages over classical treatment for micrognathia in a very specific group of patients.

Adolescent↗
PubMed1994

Mandibular distraction lengthening in the severely hypoplastic mandible: a problematic case with tongue aplasia.

Distraction lengthening is a technique that was initially developed for correction of lower limb length discrepancies. It has recently been adapted to maxillofacial problems and has gained increasing popularity in this application. This report illustrates the principles involved in mandibular distraction lengthening and offers possible solutions to potentially limiting clinical problems. First, conventional technique dictates that a distinct periosteal sleeve is necessary for distraction osteogenesis to occur. However, in this case, distraction was performed through an area of scar tissue in which the native periosteum was destroyed. The ability to extend the use of the technique of distraction successfully to suboptimal clinical situations may broaden the indications for its use. Second, fixator instability is a potentially common problem in these cases because of the forces involved in distraction osteogenesis and the duration of the process. However, mechanical rigidity is essential consolidation (typically requiring twice as long as the distraction phase). Fixator instability can be successfully salvaged during the mineralization phase of bony healing through the use of an onlay corticocancellous bone "plate," which resorbs as the distraction gap gains strength. This report highlights one of the main advantages of the distraction process: the expansion of the soft-tissue matrix of the face.

Airway Obstruction↗