• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.43 no.2
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• pp.134-137
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• 2017

Giant cell tumor (GCT) of the craniofacial bones has been reported but they are not common. This tumor occurs more often in women than in men and predominantly affects patients around the third to fifth decade of life. GCTs are generally benign but can be locally aggressive as well. We report a case of GCT involving the temporomandibular joint (TMJ), which was initially thought to be temporomandibular disorder (TMD). A 22-year-old female presented with swelling and pain over the right temporal region for 18 months associated with jaw locking and clicking sounds. On examination, her jaw deviated to the right during opening and there was a $2{\times}2$ cm swelling over the right temporal region. Despite routine treatment for TMD, the swelling increased in size. Computed tomography and magnetic resonance imaging of the brain and TMJ revealed an erosive tumor of the temporal bone involving the TMJ which was displacing the temporal lobe. Surgical excision was done and the tumor removed completely. Histopathological examination was consistent with a GCT. No clinical or radiological recurrence was detected 10 months post-surgery.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.47 no.2
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• pp.141-144
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• 2021

Osteopathia striata with cranial sclerosis (OS-CS) is a bone dysplasia characterized by a linear striated pattern of sclerosis, especially in the long bones, and cranial sclerosis. It has variable clinical findings but distinctive radiological findings. Multiple oral and dental findings have been associated with this disease and can be seen during dental and/or medical imaging of the head and neck. Dentists and clinicians must be familiar with these signs to differentiate them from pathosis or erroneous radiographs. In the following case, we present a patient with OS-CS that presented at The University of Florida College of Dentistry with multiple craniofacial manifestations of this syndrome that were seen on a panoramic radiograph, which is one of the most commonly requested radiographs by dentists.

• Archives of Craniofacial Surgery
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• v.17 no.1
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• pp.9-13
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• 2016

Background: Position of the facial foramina is important for regional block and for various maxillofacial surgical procedures. In this study, we report on anthropometry and morphology of these foramina using three-dimensional computed tomography (3D-CT) data. Methods: A retrospective review was performed for all patients who have undergone 3D-CT scan of the facial skeleton for reasons other than fracture or deformity of the facial skeleton. Anthropometry of the supraorbital, infraorbital, and mental foramina (SOF, IOF, MF) were described in relation to facial midline, inferior orbital margin, and inferior mandibular margin (FM, IOM, IMM). This data was analyzed according to sex and age. Additionally, infraorbital and mental foramen were classified into 5 positions based on the anatomic relationships to the nearest perpendicular dentition. Results: The review identified 137 patients meeting study criteria. Supraorbital foramina was more often in the shape of a foramen (62%) than that of a notch (38%). The supraorbital, infraorbital, and mental foramina were located 33.7 mm, 37.1 mm, and 33.7 mm away from the midline. The mean vertical distance between IOF and IOM was 13.4 mm. The mean distance between MF and IMM was 21.0 mm. The IOF and MF most commonly coincided with upper and lower second premolar dentition, respectively. Between the sex, the distance between MF and IMM was significantly higher for males than for female. In a correlation analysis, SOF-FM, IOF-FM and MF-FM values were significantly increased with age, but IOF-IOM values were significantly decreased with age. Conclusion: In the current study, we have reported anthropometric data concerning facial foramina in the Korean population, using a large-scale data analysis of three-dimensional computed tomography of facial skeletons. The correlations made respect to patient sex and age will provide help to operating surgeons when considering nerve blocks and periosteal dissections around the facial foramina.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.6 no.2
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• pp.94-98
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• 2010

Axenfeld-Rieger syndrome is a rare autosomal dominant disorder characterized by various ocular and extraocular malformations. The incidence of Axenfeld-Rieger syndrome has been estimated to be 1 per 200,000. The syndrome is characterized by short body stature, delayed bone age, and deficient or arrested development of neural crest cells involving the anterior chamber of the eyes, facial bones, teeth, periumbilical skin, and cardiovascular system. This is a case of a 6 year-old girl, who came to the department of the pediatric dentistry, Yonsei University Dental Hospital, for evaluation and treatment of multiple congenital missing permanent teeth. The patient presented typical dental, craniofacial, and systemic features of Axenfeld-Rieger syndrome, such as glaucoma, oval pupil, heterochromatic iris, umbilical hernia, and delayed bone age. On the panoramic view, 3 primary teeth were missing and 13 permanent tooth germs were absent. On the lateral cephalograph, underdevelopment of the maxilla and normal growth pattern of the mandible were confirmed. Periodic dental follow-up is planned for evaluation and interceptive treatment of her dental and craniofacial problems. Denture or removable space maintainer with a pontic is considered for esthetic and functional restoration. In addition, orthognathic surgery is also planned in future to prevent further midfacial skeletal deformation. Early diagnosis of Axenfeld-Rieger syndrome is very important to prevent exacerbation of complications, such as glaucoma and skeletal deformities.

• Clinical and Experimental Pediatrics
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• v.46 no.1
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• pp.91-94
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• 2003

Raine syndrome was described as an unknown syndrome in 1989. It is characterized by severe craniofacial anomalies with microcephaly, hypoplastic nose, depressed nasal bridge, exophthamos/protosis, gum hypertrophy, cleft palate, low-set ears, small mandible, narrow chest, wide cranial sutures and choanal atresia or stenosis, by generalized osteosclerosis with subperiosteal thickening of ribs, clavicles and diaphysis of long bones, and by intracranial calcifications in the particularly periventricular area. It undergoes an autosomal recessive inheritance. Twelve cases of Raine syndrome have been reported in the literature. However, a case of Raine syndrome in Korea has not been reported yet. Therefore, we describe a female newborn with Raine syndrome with a brief review of the literatures.

• Archives of Craniofacial Surgery
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• v.23 no.4
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• pp.171-177
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• 2022

Background: The treatment of zygoma complex fractures is of crucial importance in the field of plastic surgery. However, surgical methods to correct zygoma complex fractures, including the number of fixation sites, differ among operators. Although several studies have compared two-point and three-point fixation, no comparative research has yet been conducted on one-point versus two-point fixation using computed tomography scans of surgical results. Therefore, the present study aimed to address this gap in the literature by comparing surgical results between one-point and two-point fixation procedures. Methods: In this study, we randomly selected patients to undergo surgery using one of two surgical methods. We analyzed patients with unilateral zygoma complex fractures unaccompanied by other fractures according to whether they underwent one-point fixation of the zygomaticomaxillary buttress or two-point fixation of the zygomaticomaxillary buttress and the zygomaticofrontal suture. We then made measurements at three points-the zygomaticofrontal suture, inferior orbital wall, and malar height-using 3-month postoperative computed tomography images and performed statistical analyses to compare the results of the two methods. Results: All three measurements (zygomaticofrontal suture, inferior orbital wall, and malar height) showed significant differences (p< 0.05) between one-point and two-point fixation. Highly significant differences were found for the zygomaticofrontal suture and malar height parameters. The difference in the inferior wall measurements was less meaningful, even though it also reached statistical significance. Conclusion: Using three parameters in a statistical analysis of imaging findings, this study demonstrated significant differences in treatment outcomes according to the number of fixations. The results indicate that bone alignment and continuity can be achieved to a greater extent by two-point fixation instead of one-point fixation.

• The korean journal of orthodontics
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• v.33 no.6 s.101
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• pp.485-497
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• 2003

The form and function of the craniofacial structure critically depend on genetic information. With recent advances in the molecular technology, genes that are important for normal growth and morphogenesis of the craniofacial skeleton are being rapidly uncovered, shaping up modem craniofacial biology. One of them is fibroblast growth factor receptor 2 (FGFR2). Specific point mutations in the. FGFR2 gene have been linked to Apert syndrome, which is characterized by premature closure of cranial sutures and craniofacial anomalies as well as limb deformities. To study pathogenic mechanisms underlying craniosynostosis phenotype of Apert syndrome, we used a transgenic approach; an FGFR2 minigene construct containing an Apert mutation (a point mutation that substitute proline at the position 253 to arginine; P253R) was introduced into fertilized mouse germ cells by DNA microinjection. The injected cells were then allowed to develop into transgenic mice. We used a bone-specific promoter (a DNA fragment from the type I collagen gene) to confine the expression of mutant FGFR2 gene to the bone tissue, and asked whether expression of mutant FGFR2 in bone is sufficient to cause the craniosynostosis phenotype in mice. Initial characterization of these mice shows prematurely closed cranial sutures with facial deformities expected from Apert patients. We also demonstrate that the transgene produces mutant FGFR2 protein with increased functional activities. Having this useful mouse model, we now can ask questions regarding the role of FGFR2 in normal and abnormal development of cranial bones and sutures.

• The Journal of the Korean bone and joint tumor society
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• v.14 no.1
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• pp.62-67
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• 2008

Malignant degeneration of fibrous dysplasia is rare and involves transformation into osteosarcoma, fibrosarcoma and chondrosarcoma. The most frequent sites involved in malignant transformation were craniofacial bones, proximal femur, humerus, pelvis, tibia and scapula in a decreasing order of frequency. An 41-year-old man with a history of polyostotic fibrous dysplasia presented with increasing left arm pain. Plain radiograph showed expansile destructive lesion along the humeral shaft. As initial biopsy report was low grade chondrosarcoma, he underwent marginal resection. However, he developed local recurrence 7 month later and subsequent pathologic finding was chondroblastic osteosarcoma. We report one case of secondary chondroblastic osteosarcoma from polyostotic fibrous dysplasia initially misdiagnosed as low grade chondrosarcoma that caused fallacy in treatment strategy.

• Journal of Korean Academy of Oral and Maxillofacial Radiology
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• v.27 no.1
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• pp.231-241
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• 1997

The aneurysmal bone cyst is a nonmalignant reactive bone lesion. Developing rarely in the craniofacial region, and more commonly affecting the long bones and the spine, the lesion has variable etiopathogenic characteristics. The authors diagnosed a 33-year-old female as aneurysmal bone cyst after undergoing clinical, radiological and histological examinations. The characteristics were as followed: 1. The patient complained of pain and swelling of the right preauricular area. 2. The conventional radiograms showed a relatively well defined radiolucent lesion with partially scalloping margin. The cortical bone of the right condyle was thinned and expanded by the lesion. 3. Bone scintigraphy with ~c demonstrated ring-like or doughnut-pattern accumulation of radioactivity. 4. On Tl-weighted imaging of MRI, the lesion on the right condyle had middle signal intensity. T2-weighted MRI demonstrated multiple high signal intensities seperated by septa which had low signal intensity. Finger in balloon appearance was seen. 5. Histologically, the lesion was composed of large sinusoidal blood spaces lined by fibroblasts and histiocytes. Its fibrous stroma consisted of fibroblstic element, multinucleated giant cells, extravasated erythrocytes and focal hemosiderin pigmentation. New bone formation was also observed around larger sinusoidal spaces.

• Journal of the korean academy of Pediatric Dentistry
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• v.30 no.3
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• pp.391-405
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• 2003

Craniosynostosis, known as a premature fusion of cranial sutures, is a developmental disorder characterized by precocious differentiation and mineralization of osteoblasts in the calvarial sutures. Recent genetic studies have demonstrated that mutation in the homeobox gene Msx2 causes Boston-type human craniosynostosis. Additionally, the phenotype of Dlx5 homozygote mutant mouse presents craniofacial abnormalities including a delayed ossification of calvarial bone. Furthermore transcription of osteocalcin, a mature osteoblast marker, is reciprocally regulated by the homeodomain proteins Msx2 and Dlx5. These facts suggest important roles of osteocalcin, Msx2 and Dlx5 genes in the calvarial bone growth and suture morphogenesis. To elucidate the function of these molecules in the early morphogenesis of mouse cranial sutures, we have first analyzed by in situ hybridization the expression of osteocalcin, Msx2 and Dlx5 genes in the developing parietal bone and sagittal suture of mouse calvaria during the embryonic (E15-E18) stage. Osteocalcin mRNA was found in the periosteum of parietal bones from E15, and gradually more highly expressed with aging. Msx2 mRNA was intensely expressed in the sutural mesenchyme, osteogenic fronts and mildly expressed in the dura mater during the embryonic stage. Dlx5 mRNA was intensely expressed osteogenic fronts and the periostem of parietal bones. To further examine the upstream signaling molecules of transcription factor Msx2 and Dlx5, we have done in vitro experiments in E15.5 mouse calvarial explants. Interestingly, implantation of BMP2-, BMP4-soaked beads onto the osteogenic fronts after 48 hours organ culture induced etopic expressions of Msx2 and Dlx5 genes. On the other hand, overexpression of $TGF{\beta}1$, GDF-6, -7, FGF-2, -4 and Shh did not induce the expression of Msx2 and Dlx5. Taken together. these data indicate that transcription factor Msx2 and Dlx5 play critical roles in the calvarial bone and suture development, and that BMP siganling is involved in the osteogenesis of calvarial bones and the maintenance of cranial sutures through regulating these two transcriotpn factors. Furthermore, different expression patterns between Msx2 and Dlx5 suggest their specific functions in the osteoblast differentiation.