1/61. Increased occurrence of cleft lip in glycogen storage disease type ii (GSDII): exclusion of a contiguous gene syndrome in two patients by presence of intragenic mutations including a novel nonsense mutation Gln58Stop.Genetic deficiency of lysosomal acid alpha-glucosidase (acid maltase) results in the autosomal recessive disorder glycogen storage disease type ii (GSDII) in which intralysosomal accumulation of glycogen primarily affects function of skeletal and cardiac muscle. During an earlier review we noted 3 in 100 cases of GSDII with incidental description of cleft lip. In addition, we identified 2 of 35 GSDII patients referred to us for molecular studies with co-occurence of cleft lip, considerably greater than the estimated frequency of nonsyndromic cleft lip with or without cleft palate of 1 in 700 to 1,000. Because several lines of evidence support a minor cleft lip/palate (Cl/P) locus on chromosome 17q close to the locus for GSDII, we defined the molecular basis for the GSDII in these two patients to determine if they represented a contiguous gene syndrome. Patient I (of Dutch descent) was homozygous and the parents heterozygous for an intragenic deletion of exon 18 (deltaex18), common in Dutch patients. Patient II was heterozygous for delta525T, a mutation also common in Dutch patients and a novel nonsense mutation (172 [corrected] C-->T; Gln58Stop) in exon 2, the first coding exon. The mother was heterozygous for the delta525T and the father for the 172 [corrected] C-->T; Gln58Stop. The finding that both patients carried intragenic mutations eliminates a contiguous gene syndrome. Whereas the presence of cleft lip/cleft palate in a patient with GSDII could be coincidental, these co-occurences could represent a modifying action of acid alpha-glucosidase deficiency on unlinked or linked genes that result in increased susceptibility for cleft lip.- - - - - - - - - - ranking = 1keywords = dental (Clic here for more details about this article) |
2/61. Midface distraction osteogenesis in cleft patients: a case report.We present a case of midface distraction in a bilateral cleft lip and palate patient. The patient was a 10-year-old who underwent a high LeFort I osteotomy followed by placement of the Rigid External Distraction halo. Distraction was commenced on the fifth postoperative day at a rate of 1 to 1.5 mm per day until a total of 17 mm of maxillary advancement had been achieved. There were no complications and follow up was at 9 months post distraction. Results show that the patient had improved facial aesthetics and dental occlusion which was overcorrected to a Class III relationship. Velopharyngeal function was unaffected. Distraction osteogenesis of the midfacial skeleton in cleft patients offers the possibility to remodel not only the underlying bony skeleton but also all the soft tissues of the face and palate.- - - - - - - - - - ranking = 0.53856605647805keywords = dental, occlusion (Clic here for more details about this article) |
3/61. Treatment of an open-bite malocclusion complicated by clefts of the maxilla and mandible.This is a case presentation of a young girl with a severe Class II, Division I open-bite malocclusion. Her orthodontic problems were further complicated by clefts in both her maxilla and mandible. A cleft palate team evaluation brought several systemic and local problems to light which necessitated their correction prior to the commencement of any orthodontic therapy. Her diagnosis and treatment have been discussed here with special emphasis on the problems peculiar to children with oral clefts.- - - - - - - - - - ranking = 0.19283028239027keywords = occlusion (Clic here for more details about this article) |
4/61. cleft lip and palate management with maxillary expansion and space opening for a single tooth implant.An adult Class I malocclusion with a unilateral cleft lip and palate is presented. The maxillary transverse deficiency was managed with orthopedic expansion and the missing lateral incisor with space opening, bone grafting, and single tooth implant. The mild maxillary retrognathia and deficient lip support was managed with dental compensation.- - - - - - - - - - ranking = 0.53856605647805keywords = dental, occlusion (Clic here for more details about this article) |
5/61. Reconstruction of an alveolar cleft for orthodontic tooth movement.Bone grafting to repair an alveolar cleft has long been an integral part of the treatment of persons with unilateral and bilateral clefts of the lip and alveolus. The presence of the cleft places a limitation on the orthodontist who would like to move teeth in the area of the cleft. Various grafting materials have been placed in alveolar clefts in an attempt to solve this problem. The case to be presented is a patient with a Class II, Division 2, malocclusion with a left unilateral alveolar cleft and a repaired cleft lip. Ten months after initiating orthodontic treatment, a free gingival graft procedure was performed because of insufficient vestibular depth and the narrow width of the keratinized attached gingiva at the left maxillary lateral and central incisor region. Two months after periodontal surgery, a mix of decalcified freeze-dried bone allograft and a granular bioactive glass graft material (1:1) were applied subperiostally on the buccal aspect of the edentulous cleft region. Six months later, the teeth adjacent to the grafted alveolar cleft were orthodontically moved into the edentulous area. The treatment results indicated that orthodontic, periodontal, and surgical interventions resulted in a successful closure of the alveolar cleft as well as improved periodontal conditions of the teeth adjacent to the cleft area. From the orthodontic point of view, tooth movement can be achieved successfully into a bone graft made of freeze-dried bone and bioactive glass.- - - - - - - - - - ranking = 0.038566056478054keywords = occlusion (Clic here for more details about this article) |
6/61. Interdental distraction osteogenesis and rapid orthodontic tooth movement: a novel approach to approximate a wide alveolar cleft or bony defect.The closure of a wide alveolar cleft and fistula in cleft patients and the reconstruction of a maxillary dentoalveolar defect in traumatic patients are challenging for both orthodontists and surgeons. This is due to the difficulty in achieving complete closure by using local attached gingiva and the great volume of bone required for the graft. In this article, the authors propose using interdental distraction osteogenesis to create a segment of new alveolar bone and attached gingiva for the complete approximation of a wide alveolar cleft/fistula and the reconstruction of a maxillary dentoalveolar defect. They performed this procedure on one patient with a traumatic maxillary dentoalveolar defect and 10 patients with unilateral or bilateral cleft lips and palates who had varied dentoalveolar clefts/fistulas. Interdental and maxillary osteotomies were performed on one side of the dental arch by the cleft or defect. After a latency period of 3 days, the osteotomized distal segment of the dental arch was then distracted and transported toward the cleft or defect by using a toothborne intraoral distraction device. The alveoli and gingivae on both ends of the cleft or defect were approximated after distraction osteogenesis. The need for extensive alveolar bone grafting was eliminated. A segment of new edentulous alveolus and attached gingiva was created interdentally at a site distant to the cleft or defect. In the cleft patients, teeth were moved orthodontically into the regenerate (newly formed alveolar bone) dental crowding 1 week after distraction. The orthodontic tooth movement was rapidly completed in 3 months, and the edentulous space was eliminated. Interdental distraction osteogenesis minimizes an alveolar cleft/fistula and helps reconstruct a maxillary dentoalveolar defect by approximating the native alveoli and gingivae; it also creates new alveolar bone and gingiva for rapid orthodontic tooth movement.- - - - - - - - - - ranking = 5.5keywords = dental (Clic here for more details about this article) |
7/61. Dental rehabilitation using endosseous implants and orthognathic surgery in patients with cleft lip and palate: report of two cases.We describe the use of endosseous implants in the autogenous particulate cancellous bone and marrow grafted alveoli after orthognathic surgery for dental rehabilitation of patients with cleft lip and palate. This procedure has been applied to two patients and produced good results functionally and aesthetically. The results are encouraging and indicate that implant placement after orthognathic surgery is useful for patients both with congenital missing teeth and retrognathic maxillae.- - - - - - - - - - ranking = 0.5keywords = dental (Clic here for more details about this article) |
8/61. Osseointegrated implants as an adjunct to facemask therapy: a case report.Branemark Implants were placed in the zygomatic buttresses of the maxilla in a 12-year and 1-month-old female patient with a Class III malocclusion caused by maxillary growth retardation secondary to repair of a unilateral cleft lip and palate defect. The implants were left to integrate for 6 months followed by placement of customized abutments that projected into the buccal sulcus. Elastic traction (400 g per side) was applied from a facemask to the implants at 30 degrees to the occlusal plane for 14 hours per day for 8 months (ages 12 years and 10 months to 13 years and 6 months). The maxilla moved downward and forward 4 mm rotating anteriorly as it was displaced. The change in the maxillary occlusal plane resulted in a secondary opening of the mandible. There was a 2 degrees increase in the SN-mandibular plane angle and an increase in nasion to menton distance of 9 mm. Clinically, this resulted in an increase in fullness of the infraorbital region and correction of the pretreatment mandibular prognathism. There was an increase in nasal prominence as the maxilla advanced. This contributed to the increase in facial convexity. The secondary dental change frequently seen in standard facemask therapy was avoided. The displacement of the maxilla was stable 1 year beyond cessation of facemask therapy. The patient's midface profile was improved by age of 13 years and 6 months. Details of the clinical procedure and treatment changes are presented.- - - - - - - - - - ranking = 0.53856605647805keywords = dental, occlusion (Clic here for more details about this article) |
9/61. Orthodontic and bone grafting procedures in a cleft lip and palate series: an interim cephalometric evaluation.An attempt is being made to observe the facial and dental development of a cleft lip and palate sample. The study is continuing and, hopefully, offers insight into what happens to these children who have undergone the placement of a dentofacial maxillary orthopedic appliance early at the time of lip closure and an additional surgical procedure and autogenous osteoplasty. Though the final answers to the two previously posed questions are not yet available, we are able to make some value judgments by observing the lateral cephalometric films of these children, and observing their occusion intraorally. Thus far, we are able to state that in our sample, using our treatment procedures in the sequence advocated, we have seen no growth attenuation in the posterior/anterior dimension. The maxilla, at least to the ages observed, does not appear to have been attenuated by our procedures. Further, it would seem that after a limited first phase of orthodontic treatment to align dental units, the degree of crossbite is considerably smaller from that reported in the literature by those using more conventional approaches; thus it is possible that we are doing some good. We are still using these procedures on our newborn and continue to feel that we have a real opportunity to be able to do more orthodontically for these children when they possess a full, permanent dentition and are ready for comprehensive treatment.- - - - - - - - - - ranking = 1keywords = dental (Clic here for more details about this article) |
10/61. Description of a clinical technique for tooth extraction in the cleft lip and palate area.cleft lip and palate are relatively common congenital malformations, which may require specialist paedodontic treatment. In this article, the case of a 9-year-old boy with bilateral complete cleft lip and palate is presented. He attended the Hospital for rehabilitation of Craniofacial Anomalies (HRAC) for routine examination, during which the presence of pre-canine supernumeraries bilaterally in the cleft area was seen. The extraction of these dental elements was justified by extensive carious lesions and because they represented a potential problem during secondary palatoplasty. The precautions needed in tooth extraction in patients with cleft lip and palate are described, together with illustrations of the clinical procedure.- - - - - - - - - - ranking = 0.5keywords = dental (Clic here for more details about this article) |
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