1/96. Computer-aided surgery in distraction osteogenesis of the maxilla and mandible.When using unidirectional intraoral distraction devices, it is desirable to be able to determine the final position of the bone fragment after the distraction procedure. However, additional constraining forces from adjacent tissues render the prediction of the distraction direction difficult. We have utilised computer-aided surgery in three patients for intraoperative control of the distraction direction. In one cleft palate patient, suffering from maxillary hypoplasia and anterior open bite, a modified Le Fort I osteotomy and maxillary distraction was performed. Despite a ventrocaudal position of the distraction device, intraoperative computer visualisation showed an unfavourable caudal vector of distraction without any anterior movement. The final result confirmed the direction indicated by the computer. Maxillary advancement remained insufficient. In two patients suffering from mandibular hypoplasia, intraoperative assessment revealed a favourable direction of distraction. The distraction procedure led to a satisfactory result in both cases. Computer-aided surgery is helpful in assessing the vector of distraction intraoperatively, making the result of the distraction procedure more predictable and allowing instant correction by adequate reapplication of the device.- - - - - - - - - - ranking = 1keywords = mandible (Clic here for more details about this article) |
2/96. Results with sphincter pharyngoplasty and pharyngeal flap.OBJECTIVE: To evaluate speech outcomes and complications of sphincter pharyngoplasty and pharyngeal flap performed for management of velopharyngeal insufficiency (VPI). DESIGN: Case series. SETTING: Tertiary care children's hospital. patients: All patients who underwent pharyngeal flap or sphincter pharyngoplasty from 1990 to 1995. methods: Perceptual speech analysis was used to assess severity of VPI, presence of nasal air emissions and quality of nasal resonance (hyper, hypo, or normal). Pre-operative measures of velopharyngeal function were based upon nasendoscopy and videofluoroscopic speech assessment. Recommendations for management were made by the attending surgeon. Complications of hyponasality and obstructive sleep symptoms (OSS) were noted. Patient characteristics were compared using univariate analysis. RESULTS: Sixteen patients underwent sphincter pharyngoplasty and 18 patients underwent superiorly based pharyngeal flap. patients were similar in terms of lateral pharyngeal wall medial motion and palatal elevation. The groups were also similar with regard to VPI severity, though there was a trend for more severe VPI in patients undergoing sphincter pharyngoplasty than pharyngeal flap (50 vs. 33.3%, respectively). patients with pharyngoplasty had a higher rate of resolution of VPI than those who had pharyngeal flap (50 vs. 22.2%, respectively), although this was not statistically significant. Post-operative hyponasality and obstructive sleep symptoms were present in both groups. However, only patients who underwent PF and had postoperative OSS had obstructive sleep apnea (OSA). CONCLUSIONS: There were no detectable anatomic differences between treatment groups implying that treatment selection during the study period was not guided by strict anatomic criteria. Sphincter pharyngoplasty may have a higher success rate with a lower risk of OSS.- - - - - - - - - - ranking = 0.016964524281128keywords = lower (Clic here for more details about this article) |
3/96. Blepharo-cheilo-dontic (BCD) syndrome in two Mexican patients.The combination of lagophthalmia, ectropion of the lower eyelids, distichiasis, euryblepharon, cleft lip/palate, and oligodontia was recently named blepharo-cheilo-dontic (BCD) syndrome. Different combinations of these signs have been found sporadically, with autosomal dominant inheritance. ectropion of the lower eyelids, lagophthalmia, and bilateral cleft lip/palate appear to be the more common manifestations. We report on two unrelated patients with bilateral cleft lip/palate and lagophthalmia. One of these two patients had familial cleft lip/palate in two generations, probably as a variable expression of an autosomal dominant gene.- - - - - - - - - - ranking = 0.033929048562257keywords = lower (Clic here for more details about this article) |
4/96. Popliteal pterygium syndrome: a clinical study of three families and report of linkage to the Van der Woude syndrome locus on 1q32.Popliteal pterygium syndrome (PPS) is a rare autosomal dominant disorder, thought to occur with an incidence of approximately 1 in 300 000 live births. The main clinical manifestations are popliteal webbing, cleft lip, cleft palate, lower lip pits, syndactyly, and genital and nail anomalies. This report describes the clinical features in two families with PPS and one isolated case, showing the range of anomalies found both within and between the families. PPS has some features in common with Van der Woude syndrome (VWS), also inherited as an autosomal dominant condition, with cleft lip/palate and, more distinctively, lower lip pits. Although the gene for VWS has not yet been identified, it has been localised to within 1.6 cM in the region 1q32-41. To determine whether PPS and VWS represent allelic forms of the same gene, three families were genotyped for markers flanking and within the critical region. A multipoint lod score of 2.7 was obtained, with no evidence of recombination, supporting the hypothesis that these two disorders are allelic.- - - - - - - - - - ranking = 0.033929048562257keywords = lower (Clic here for more details about this article) |
5/96. 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 = 1.25keywords = mandible (Clic here for more details about this article) |
6/96. Facial growth in a cleft palate patient treated with the Herbst appliance: a long-term profile roentgenographic and roentgen stereometric analysis of profile changes and displacement of the jaws.OBJECTIVE: To monitor facial development in a patient with cleft palate who was treated with the Herbst appliance. Monitoring was in terms of changes in the skeletal profile and growth in the circummaxillary sutures and temporomandibular joints (TMJs). DESIGN: Prospective profile roentgenography (between the ages of 6 and 20 years) and roentgen stereometric analysis (between the ages of 8 and 19 years). SETTING: Center for Craniofacial Anomalies and Department of Plastic and Reconstructive Surgery, Malmo University Hospital, Malmo, sweden. PATIENT: Boy with cleft of the soft and posterior part of the hard palate and marked facial convexity. INTERVENTIONS: Surgical repair of the soft palate at age 9 months, velopharyngeal flap at age 8 years, and insertion of implants under general anesthesia and treatment with the Herbst appliance at age 11 years. Roentgen examinations were performed in connection with continued clinical evaluations and treatment. MAIN OUTCOME MEASURES: Profile roentgenograms were traced and measured by one of the authors using conventional point-based analysis. Stereo roentgenograms were digitized by the Department of Orthopedic Surgery, Malmo University Hospital. RESULTS: The direction of profile changes was partly discordant with the direction of articular growth in the circummaxillary sutures and TMJs. The successful treatment result was accomplished by a temporary influence on sagittal growth direction in the circummaxillary sutures and on rotational growth direction in the TMJs, combined with a favorable natural remodeling and articular growth pattern. CONCLUSIONS: The mode of growth by which treatment aims were reached was partly unexpected, i.e., discordant with the generally accepted principal concept that treatment with the Herbst appliance positions the mandible forward.- - - - - - - - - - ranking = 0.65812034697084keywords = mandible, jaw (Clic here for more details about this article) |
7/96. Prenatal ultrasonographic diagnosis of the popliteal pterygium syndrome.The prenatal ultrasound identification of a cleft lip and palate, equinovarus feet with severe lower limb malposition and genital abnormalities led to the prenatal diagnosis of popliteal pterygium syndrome in a pregnant mother suspected to have a mild expression of this autosomal dominant condition. However, in sporadic cases with lack of a family history for this rare syndrome, prenatal diagnosis may be difficult to ascertain.- - - - - - - - - - ranking = 0.016964524281128keywords = lower (Clic here for more details about this article) |
8/96. Popliteal pterygium syndrome in a Swedish family--clinical findings and genetic analysis with the van der Woude syndrome locus at 1q32-q41.The present study describes a Swedish family in which the mother and her son were affected with signs of popliteal pterygium syndrome (PPS, OMIM 119500). Both individuals had bilateral complete cleft lip and palate, oral synechiae, paramedian pits on the lower lip, toe syndactyly and a piece of triangular skin overgrowth on the great toes. The son also presented with soft tissue syndactyly of the 2nd and 3rd fingers. Although popliteal pterygium was not found, the above clinical features were diagnostic for PPS. Chromosomal abnormalities were not revealed in either case by cytogenetic analyses. A test for microdeletion in the VWS region at 1q32-q41 was performed in the family using 5 polymorphic microsatellite markers from the region. The affected son was found to be heterozygous for all 5 markers, suggesting that microdeletion at the VWS region was unlikely. The VWS locus, however, was not excluded by haplotype analysis of the family.- - - - - - - - - - ranking = 0.016964524281128keywords = lower (Clic here for more details about this article) |
9/96. 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 = 0.25keywords = mandible (Clic here for more details about this article) |
10/96. Bilateral cleft lip and palate and limb deformities: a presentation of amniotic band sequence?We report a case of a child born with bilateral complete cleft lip and palate, absence of the left lower leg, complete absence of the right arm, and absence of the second and third distal phalanges of the right foot. The child had no other associated abnormalities. Prenatal ultrasonography recognized the cleft lip and palate but failed to appreciate the limb deformities. Although this specific constellation of abnormalities has not been previously reported in the English literature, it may represent an amniotic band sequence or two unrelated anomalies. However, unlike the vast majority of patients with amniotic band sequence described, there were no associated bizarre facial clefts or cranial defects. The case history and a review of the literature will be presented, with emphasis placed on categorization of these specific anomalies.- - - - - - - - - - ranking = 0.016964524281128keywords = lower (Clic here for more details about this article) |
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