1/21. Tessier type VI-VII cleft combination associated with congenital bimaxillary fusion and anophthalmia.Congenital intermaxillary fusion is a rare anomaly. Combination of the anomaly with any type of facial cleft is extremely rare. death in a majority of these patients as a result of feeding and aspiration problems in early life may have caused the reports to be limited. In this article a 5-year-old patient, probably the first in the literature having Tessier type VI-VII facial cleft combination associated with bimaxillary fusion and anophthalmia on the right side, is presented. The patient has survived on fluid meal through a very small opening for 5 years. The features of the case are presented, and the time and method of the management of such a rare anomaly are discussed with a review of the literature.- - - - - - - - - - ranking = 1keywords = cleft (Clic here for more details about this article) |
2/21. The Richieri-Costa and Pereira form of acrofacial dysostosis: first case in a non-Brazilian infant.We report on a French boy with cleft mandible, pre/postaxial hand anomalies, and clubfoot born to consanguineous parents. These findings are comparable to those of previous cases of the autosomal recessive Richieri-Costa and Pereira syndrome of short stature, Robin sequence, cleft mandible, pre/postaxial hand anomalies, and clubfoot. This is the first case in a non-Brazilian infant.- - - - - - - - - - ranking = 0.33333333333333keywords = cleft (Clic here for more details about this article) |
3/21. 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 = 2.2638773495091keywords = cleft palate, cleft, palate (Clic here for more details about this article) |
4/21. Transmission of the dysgnathia complex from mother to daughter.We report the first observation of parent-to-child transmission of dysgnathia, a rare disorder characterized by severe mandibular hypoplasia or agenesis, ear anomalies, microstomia, and microglossia. Patient 1 was noted prenatally by ultrasound to have severe micrognathia and, after birth, abnormal ears with canal stenosis and non-contiguous lobules located dorsally to the rest of the pinnae, normal zygomata, severe jaw immobility and microstomia with an opening of only 4 to 5 mm, hypoplastic tongue, and cleft palate. The 21-year-old mother of patient 1 was born with severe micrognathia requiring tracheostomy, microglossia, cleft palate with filiform alveolar bands, abnormal pinnae, and decreased conductive hearing. Dysgnathia is thought to result from a defect in the development of the first branchial arch. A similar phenotype has been seen in Otx2 haplo-insufficiency and endothelin-1 homozygous null mice, suggesting that these genes contribute to branchial arch development. Our report of a long-surviving mother and her daughter with non-syndromal dysgnathia may lead to identification of the molecular basis of these findings and provide insight into the genetics of first branchial arch formation. The survival of patient 1 and patient 2 beyond the neonatal period has implications for improvements in prenatal diagnosis and counseling and for neonatal treatment of this condition.- - - - - - - - - - ranking = 2.5277546990183keywords = cleft palate, cleft, palate (Clic here for more details about this article) |
5/21. "Distraction" of grafted alveolar bone in cleft case using endosseous implant.OBJECTIVE: Presentation of a specific surgical technique of vertical alveolar ridge distraction performed on an alveolar bone graft 1 year after the primary grafting procedure designed to correct a labioalveolar cleft. The graft had not obtained the results desired and presented heavily scarred mucosal tissue. TECHNIQUE: A dental implant placed within the graft once it has been entirely freed by osteotomies functions as an endless screw. To avoid vascular risk, the alveolar bone graft is detached as a whole along its total height. Consequently, no bone remains above it to provide support for a conventional alveolar distractor. An osteosynthetic miniplate, fastened in a horizontal position beneath the nasal mucosae above the graft, is used to replace the missing bony support and to stabilize the implant, which activates the distraction process. PATIENT: This study is based on our first clinical case with a follow-up of 18 months at present. RESULT: A vertical displacement of the entire graft was achieved, including its attached mucosal layer, which repositioned both bone and mucosa on a far better level in the cleft area. Thus, a normal length of abutment was obtained permitting prosthetic rehabilitation based on the same endosseous implant, which was left in place within the graft. CONCLUSION: This technique may prove particularly helpful in certain cases in which a primary alveolar bone grafting procedure has produced borderline results. In such cases, on the one hand, neither the reasonably satisfactory volume of the bone graft itself nor the poor quality of its scarred mucosal tissue argue in favor of a secondary grafting procedure. On the other hand, it is impossible to resort to currently available alveolar distracters since our choice of techniques leaves no bone support above the graft.- - - - - - - - - - ranking = 1keywords = cleft (Clic here for more details about this article) |
6/21. Seventeen-year follow-up of a patient with median cleft of the lower lip, mandible, and tongue with flexion contracture: a case report.PURPOSE: We present a 17-year follow-up of an unusual patient with median cleft of the lower lip, mandible, and tongue with a flexion contracture. CONCLUSION: Timing of the mandibular reconstruction needs to take into account growth of the mandible and masticatory function. In this case, fixation of the mandible performed at the age of 6 years 6 months and reconstruction carried out at 7 years 8 months of age resulted in satisfactory occlusion and masticatory function.- - - - - - - - - - ranking = 0.83333333333333keywords = cleft (Clic here for more details about this article) |
7/21. Popliteal pterygium syndrome with special consideration of the cleft malformation: case report.This report describes a new case of popliteal pterygium syndrome (PPS) and also a treatment protocol. The patient presented with the complete complex of PPS and additional abnormalities that have not been described in the literature: a sinus of the upper lip, an extreme hypopoplastic prolabium with aplasia of the vestibule in this area, and a velar pterygium.- - - - - - - - - - ranking = 0.66666666666667keywords = cleft (Clic here for more details about this article) |
8/21. Effects of orthognathic surgery on nasal form and function in the cleft patient.Management of common problems of nasal airway obstruction in cleft and noncleft patients by the subnasal approach through the maxillary Le Fort I osteotomy are discussed. The effects of maxillary surgical repositioning on the esthetics of the nose and upper lip are presented, as well as the sequencing and timing of orthognathic surgery and nasal reconstruction in the cleft patient. Case presentations illustrate the results of this treatment approach.- - - - - - - - - - ranking = 1.1666666666667keywords = cleft (Clic here for more details about this article) |
9/21. Rigid external distraction using skeletal anchorage to cleft maxilla united with alveolar bone grafting.OBJECTIVE: documentation of the application of maxillary distraction osteogenesis using rigid external distraction (RED) with skeletal anchorage combined with predistraction alveolar bone grafting (ABG) in cleft maxilla. DESIGN: Case report. PATIENT: A patient with numerous congenital missing teeth and severe maxillary deficiency related to complete bilateral cleft lip and palate with large alveolar bone defect. INTERVENTION: The patient received preoperative orthodontic treatment, predistraction ABG, and maxillary distraction osteogenesis using RED with skeletal anchorage. RESULTS: Predistraction ABG completely united the cleft maxilla. The united maxilla was successfully advanced by the RED system with skeletal anchorage, despite unsound dentition with numerous congenital missing teeth. CONCLUSION: The present study demonstrates that the combination of predistraction ABG and RED system with skeletal anchorage is effective for the treatment of severe maxillary deficiency related to complete bilateral cleft lip and palate with large bone defect and numerous congenital missing teeth.- - - - - - - - - - ranking = 1.4981854139024keywords = cleft, palate (Clic here for more details about this article) |
10/21. pycnodysostosis. Report of a case and review of the Japanese literature, with emphasis on oral and maxillofacial findings.We report a case of pycnodysostosis and review 54 cases of this syndrome in the Japanese literature, with special emphasis on oral and maxillofacial findings. Common findings were as follows: hypoplasia of maxilla and mandible, hypopneumatization of the maxillary sinuses, loss of mandibular angle, a grooved palate, and malpositioned teeth.- - - - - - - - - - ranking = 0.082426040284521keywords = palate (Clic here for more details about this article) |
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