Cases reported "Synostosis"

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1/10. Musculoskeletal manifestations of the Antley-Bixler syndrome.

    The Antley-Bixler syndrome is a rare disorder with many musculoskeletal anomalies that demand orthopedic assessment. The syndrome includes skeletal, craniofacial, and urogenital anomalies. The most common skeletal deformities are radiohumeral synostosis, craniosynostosis, multiple joint contractures, and arachnodactyly. Other orthopedic manifestations that may occur are femoral bowing, ulnar bowing, camptodactyly, synostoses of carpal and tarsal bones, clubfoot, vertebral body anomalies, perinatal fractures, and advanced skeletal age. The inheritance pattern is thought to be autosomal recessive. A patient with this syndrome is described, which is the 18th of 24 reports published in the world literature. This case is compared with the other reported cases.
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ranking = 1
keywords = craniofacial
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2/10. Complete bony fusion of the mandible to the zygomatic complex and maxillary tuberosity: case report and review.

    Congenital craniofacial disorders represent approximately 20% of all birth defects. One of these disorders is syngnathia, of which only 24 cases have been reported since 1936. Twenty cases involved fusion of the alveolar processes of the maxilla and mandible. Only four are similar to the presented case, which includes bony fusion of the ascending ramus of the mandible to the zygomatic complex and the posterior part of the maxilla. This case report will present details from the 23rd week of gestation to 8 months of age when the infant underwent the first attempt to free the syngnathia. The literature is discussed and a causative mechanism and new classification are proposed.
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ranking = 1
keywords = craniofacial
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3/10. Craniofacial characteristics of klippel-feil syndrome in an eight year old female.

    A female eight year, one month old patient with klippel-feil syndrome has been introduced. General appearance of the patient was characterized by short neck with limited head movements, craniofacial asymmetry, low posterior hairline and a short stature. Cephalometric analysis revealed a Class I dentoskeletal pattern with an excessive mandibular plane angle and fused cervical vertebrae. Panoramic radiogram showed congenitally missing lower second premolars and right central incisor.
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ranking = 1
keywords = craniofacial
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4/10. Nager syndrome (preaxial acrofacial dysostosis): a case report.

    The Nager syndrome is a rare condition associated with craniofacial malformations such as micrognathia, zygomatic hypoplasia, cleft palate, and preaxial limb deformities. This report features a case of the Nager syndrome occurring in a 4-year-old boy showing microdontia, thumb duplication and radioulnar synostosis, and ventricular septum defect, characteristics not usually encountered in the published cases.
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ranking = 1
keywords = craniofacial
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5/10. Resorbable plate-screw systems: clinical applications.

    OBJECTIVE: To evaluate the results we obtained from 37 patients referred to our clinics with resorbable plate-screw systems which have been claimed as biodegradable fixation materials and used in craniofacial, maxillofacial and orthopedic reconstructive procedures owing to their advantages such as biocompatibility, adequate biomechanical resistance, longer dwelling time, elimination through physiological routes without causing any foreign body reaction and/or significant sequaleae. methods: Resorbable plate-screw systems used in 37 patients between 2000-2003 for various craniofacial reconstructive procedures were evaluated as for their efficacy, and biocompatibility. RESULTS: Adequate fixation was obtained in all patients, excluding one case operated for mandibula fracture whose reconstruction was completed with titanium plate-screw system. No serious complication was seen except a granuloma and sinus formation on subciliary incision in one patient. CONCLUSION: Owing to their improved polylactic acid/polyglycolic acid ratio resorbable plate-screw systems which contain varying compositions of polylactic acid and polyglycolic acid copolymers are ideal fixation materials used favourably in maxillofacial, craniofacial and orthopedic reconstructive surgery in that they make effective fixation and have further advantages such as biocompatibility, adequate biomechanical resistance against distraction and compression forces in the early postoperative period, longer dwelling time and elimination from the body through physiological routes without causing any foreign body reaction or significant sequaleae.
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ranking = 3
keywords = craniofacial
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6/10. Fetal alcohol syndrome: report of one case.

    A male premature baby, whose mother was an alcoholic, was diagnosed as a case of fetal alcohol syndrome according to the following: (1) early-onset intrauterine growth retardation and persistent postnatal growth failure; (2) psychomotor retardation; (3) craniofacial dysmorphism; (4) tetralogy of fallot; and (5) radioulnar synostosis. The literatures on the clinical manifestation, pathogenesis, and outcome of fetal alcohol syndrome are reviewed.
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ranking = 1
keywords = craniofacial
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7/10. External cranial vault molding after craniofacial surgery.

    Many congenital cranial vault deformities are only incompletely corrected by currently available surgical techniques. The supplementary use of external cranial vault molding devices after these surgical techniques, however, has resulted in consistently improved cranial vault from over what could be achieved by operation alone. These devices are inexpensive, easily individualized to abnormal skull shapes, and well tolerated by young patients.
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ranking = 4
keywords = craniofacial
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8/10. Cephalometric radiography and computed tomography in infants undergoing major craniofacial surgery--a comparison.

    Craniofacial growth after surgery in children is not completely understood. We have therefore formed a programme for pre- and postoperative studies with both roentgencephalometric- and computed tomographic investigations. This regimen results in overlapping information. Thus, an assessment of the advantages and disadvantages of the two methods was made and exemplified in four patients. In our experience both cephalometry and CT are essential for the diagnosis, surgical planning and follow-up of surgically treated children with craniofacial anomalies. To minimize the radiation doses and the diagnostic procedures which in these cases often includes general anesthesia, patients with isolated, asymmetrical conditions such as plagiocephaly, might be followed with CT only, since roentgencephalometry can not add much information.
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ranking = 5
keywords = craniofacial
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9/10. Antley-Bixler syndrome: a disorder characterized by congenital synostosis of the elbow joint and the cranial suture.

    The Antley-Bixler syndrome is a rare disorder characterized by craniosynostosis, midface hypoplasia, radiohumeral synostosis, joint contractures, arachnodactyly, and femoral bowing and fractures. We report four cases with this disorder, all of which had craniosynostosis, midface hypoplasia with characteristic facial appearance, and contractures of bilateral elbow joints. However, femoral bowing, fractures, and arachnodactyly were not seen in our patients. In addition, proximal phalanges of the thumb and the great toe showed deformity of the delta phalanx in two cases. Characteristic features in these cases were the synostotic deformity of the elbow joint; three had radioulnahumeral synostosis, and one had radioulnar synostosis. Therefore, our cases indicated that various synostotic patterns of the elbow joints may exist in this syndrome. It is reasonable to propose that characteristic craniofacial appearance associated with the synostosis of the elbow joints of various forms should be considered minimal diagnostic criteria of the Antley-Bixler syndrome.
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ranking = 1
keywords = craniofacial
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10/10. Skeletal analysis of craniofacial asymmetries in plagiocephaly (unilateral coronal synostosis).

    Why do the craniofacial bones grow asymmetrically in patients with plagiocephaly (unilateral coronal synostosis)? We obtained three-dimensional skeletal replicas of two patients with the condition and analysed the deformities of the facial bones. From this analysis we deduced that the asymmetric deformation of the facial bones in these patients was caused by a combination of three rotations: rotation of the calvaria toward the affected side because of premature synostosis of the coronal and sphenofrontal sutures; rotation of the facial bones on the horizontal plane toward the unaffected side caused by anterior displacement of the TM (temporomandibular)-joint on the affected side; and downward rotation of the facial bones toward the unaffected side caused by inferior displacement of the TM-joint on the affected side.
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ranking = 5
keywords = craniofacial
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