Cases reported "Cleft Palate"

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1/29. Dentoalveolar growth inhibition induced by bone denudation on palates: a study of two isolated cleft palates with asymmetric scar tissue distribution.

    OBJECTIVE: This report presents two cases of isolated cleft palate with asymmetric distribution of postsurgical scar tissue determined by laser Doppler flowmetry. To determine the effect of mucoperiosteal denudation of the bone on maxillary alveolar growth, the analysis of dentoalveolar structures compared the affected side to the unaffected side of each case. METHOD: Two Japanese girls with isolated cleft palates were examined. Both subjects had undergone pushback operations (a modified version of the procedure of Wardill) for palatal repair at 18 months of age. Palatal blood flow was examined by laser Doppler flowmetry when the girls were 12 years old to determine the extent of postsurgical scar tissue over the denuded bone. To analyze the maxillary dentoalveolar structures three dimensionally, the whole surface of the upper dental cast was measured and recorded by an optical measuring device when the girls were 7 years old. RESULTS AND CONCLUSIONS: Analysis via flowmetry showed that the palatal scar tissue area was limited to the anterior tooth region on the right (unaffected) side but extended posteriorly to the premolar region on the left (affected) side in both subjects. The two girls had similar dentoalveolar structures, with the dental and alveolar arches deflected lingually at the deciduous molar area on the affected side. There were no differences in the buccolingual inclination of deciduous molars or in the vertical growth of the alveolar processes between the affected and unaffected sides. In both girls, bone denudation in the premolar region appeared to result in less than 3 mm of displacement of the teeth palatally, with no change in lingual inclination. Any effects of scar tissue on the vertical development of the alveolus were not substantiated.
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2/29. Ectrodactyly, ectodermal dysplasia and cleft palate (EEC syndrome). Report of a family and review of the literature.

    A family is described in which a father and son are affected with ectrodactyly, ectodermal dysplasia and cleft palate. This particular constellation of major malformations may constitute a variant form of the EEC syndrome which characteristically includes cleft lip with or without cleft palate. From a review of the cases previously reported in the literature, autosomal dominant inheritance is the most likely mode of transmission of these conditions.
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3/29. Orthodontic treatment for jaw deformities in cleft lip and palate patients with the combined use of an external-expansion arch and a facial mask.

    patients with cleft lip and palate can suffer from contraction of the maxillary arch and anterior cross-bite accompanied by skeletal growth retardation. We use an appliance called an external-expansion arch and induce maxillary protraction using a facial mask in order to correct the anterior cross-bite and maxillary retrusion. In this paper, the method of application of these appliances and the effects of this therapy are reported here. The external-expansion arch consists of a labial wire, bands and a sectional arch. The 0.045-inch stainless steel wire extends along the maxillary dental arch. Hooks are soldered immediately distal to the lateral incisor and the distal leg of the vertical loop. The brackets are bonded to the maxillary anterior teeth, and a 0.016 x 0.016 inch sectional arch is set. The external-expansion arch is inserted into the headgear tube and ligated with the sectional arch using elastic thread. The maxillary bone is pulled by use of the facial mask and the elastic band. For traction, the force is about 300 g on each side, applied parallel to the occlusal plane or slightly downward. The duration of use is 8 to 12 hours per day. The external-expansion arch has several advantages: it can be applied from the early period of Hellman's dental age IIIA or IIC to improve anterior cross-bite. As it is easy to expand the anterior teeth and move individual teeth to the labial and buccal sides, establishment of a dental arch from severe collapse is not difficult. When an expanding device such as the Quad-helix is incorporated, lateral expansion becomes easier. Furthermore, it is easy to control the teeth vertically, and patient compliance is not necessary. Hence, this method is effective as a phase 1 treatment for orthodontic patients with cleft lip and palate characterized by maxillary retardation.
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4/29. Use of distraction osteogenesis in cleft palate patients.

    Distraction osteogenesis (DO) has been used recently to correct maxillary hypoplasia with predictable and stable results. In patients with clefts of the secondary palate, DO can also be used to aid in vertical alveolus augmentation and rapid orthodontic tooth movement. If an osteotomized dental arch can be transported to a new position without complications, it would reduce or eliminate the need for a secondary bone graft to the cleft alveolus in cleft patients and help prevent dentoalveolar defects by approximating the native alveolar bone and gingiva. Mobilizing a segment in the dentoalveolar region also results in the creation of new bone and attached gingiva. This report shows that the application of DO for skeletal expansion and rapid movement of tooth-bone segments should receive more careful consideration in the treatment of patients with clefts of the palate.
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5/29. Maxillofacial intraoral distraction osteogenesis followed by elastic traction in cleft maxillary deformity.

    We present a case of severe maxillary hypoplasia in a 16 years old cleft patient treated by distraction osteogenesis maxillary advancement. Initial evaluation showed vertical and antero-posterior maxillary deficiencies, and a Class III malocclusion. Two intraoral distractors (Zurich Pediatric Maxillary Distractor, KLS Martin, Tuttlingen, germany) were placed in a high Le Fort I osteotomy. An initial advancement of 11 mm was obtained, but the resulting occlusion was unsatisfactory (end-to-end occlusion). The consolidation period was reduced to 3 weeks to allow the mechanical manipulation of the newly formed bone with Class III elastics. An additional advancement of 3 mm, caused by elastic orthodontic traction produced both normal skeletal relationship and satisfactory occlusion. This observation shows that it is possible to carry on a skeletal maxillary displacement by interdental elastics before the complete fusion of the callus. After 12 months of postoperative follow-up no osseous relapse could be detected and the occlusal result was stable.
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6/29. The contribution of electromyography to the diagnostics of some rare palatal anomalies.

    The paper presents the electromyographic (EMG) findings of the soft palate in three patients: a patient with Mohr syndrome and cleft palate, a patient with palatal asymmetry and rhinolalia and a patient with vertical oro-ocular facial cleft with marked asymmetry of the cleft palate. In the first patient, electrical silence was registered in one half of the palate. In the second patient, moderate loss of active motor units was registered in the hypoplastic part of the palate. In the third patient, in spite of asymmetry, the EMG finding was normal on both sides of the palate.
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7/29. Experience with the functional cleft lip repair.

    The first 12 functional cleft lip repairs performed on unselected consecutive patients immediately following the completion of training by the author are presented. Previous reports on this cleft lip repair have shown excellent results but have always been based on patients operated on by the originator of the procedure. This report gives credence to the ease with which a cleft lip repair that gives reproducible good results can be taught and learned even by plastic surgeons with limited experience. It reviews the technical steps of the procedure, which emphasizes wide undermining and release of the orbicularis oris muscle on the lateral side of the cleft to allow redraping and lengthening of the lip skin, step-by-step layered closure of the mucosa, muscle, and skin, and further vertical lengthening of the lip with a Z-plasty skin closure. Three elements that are difficult to achieve or restore with cleft lip revision are evaluated: (1) achievement of a good skin scar, (2) maintenance of the alar-facial groove, and (3) achievement of adequate lip height without sacrificing horizontal lip length. Ten of the 12 patients had a satisfactory scar, 9 patients had a good alar-facial groove, and all patients had a normal-appearing horizontal lip length. Nine patients required secondary surgery; however, in six patients, this included correction of the nasal deformity that was not corrected at the time of cleft lip repair.
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8/29. Di George anomaly and velocardiofacial syndrome.

    The velocardiofacial syndrome is an autosomal dominant disorder characterized by cleft palate, cardiac anomalies, characteristic facies, and learning disabilities. The Di George anomaly involves developmental defects of the third and fourth pharyngeal pouches, resulting in thymic and parathyroid hypoplasia and cardiac defects. The cases of individuals in two families help substantiate the notion that the Di George anomaly occurs as a feature of the velocardiofacial syndrome. The proband in family 1 was a male infant with persistent hypocalcemia and cardiac defects consisting of truncus arteriosus, atrial septal defect, ventricular septal defect, and abnormal aortic arch vessels. autopsy revealed absence of thymic and parathyroid tissue, and the Di George anomaly was diagnosed. His father had a submucous cleft palate, T cell dysfunction, and facial features consistent with the velocardiofacial syndrome. This is the third case of male-to-male transmission of velocardiofacial syndrome. The proband of family 2 was a 4-year-old girl with developmental delay, persistent neonatal hypocalcemia, ventricular septal defect, T cell dysfunction, and facial features of the velocardiofacial syndrome. The Di George anomaly has been reported to occur in at least 18 different disorders. The observation that the Di George anomaly is a component manifestation of the velocardiofacial syndrome in these two families provides further evidence that the Di George anomaly is not a distinct syndrome of a single origin but rather a heterogeneous developmental field defect. It is proposed that all previously reported cases of autosomal dominant Di George anomaly are examples of the velocardiofacial syndrome.
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9/29. cleft palate and multiple anomalies in one of two siblings with partial 13 trisomy.

    siblings with multiple congenital anomalies secondary to familial partial trisomy of chromosome number 13 are described. In addition to other findings, the younger child exhibited a cleft of the soft palate. The mechanism of transmission of the chromosomal abnormalities, the relationship of the physical abnormalities and the chromosome findings, and the significance of these findings in the consideration of recurrence risks in future pregnancies are discussed.
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10/29. association of syndactyly, ectodermal dysplasia, and cleft lip and palate: report of two sibs from turkey.

    Two Turkish sibs, products of a second cousin marriage, with tetramelic syndactyly, ectodermal dysplasia, cleft lip and palate, renal anomalies, and mental retardation are reported. Similarities between these two brothers and previously reported cases and their mode of transmission are discussed.
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