• Clinical and Experimental Pediatrics
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• v.59 no.sup1
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• pp.14-18
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• 2016

Pediatric epilepsy can be caused by various conditions, including specific syndromes. 1p36 deletion syndrome is reported in 1 in 5,000-10,000 newborns, and its characteristic clinical features include developmental delay, mental retardation, hypotonia, congenital heart defects, seizure, and facial dysmorphism. However, detection of the terminal deletion in chromosome 1p by conventional G-banded karyotyping is difficult. Here we present a case of epilepsy with profound developmental delay and characteristic phenotypes. A 7-year-and 6-month-old boy experienced afebrile generalized seizure at the age of 5 years and 3 months. He had recurrent febrile seizures since 12 months of age and showed severe global developmental delay, remarkable hypotonia, short stature, and dysmorphic features such as microcephaly; small, low-set ears; dark, straight eyebrows; deep-set eyes; flat nasal bridge; midface hypoplasia; and a small, pointed chin. Previous diagnostic work-up, including conventional chromosomal analysis, revealed no definite causes. However, array-comparative genomic hybridization analysis revealed 1p36 deletion syndrome with a 9.15-Mb copy loss of the 1p36.33-1p36.22 region, and fluorescence in situ hybridization analysis (FISH) confirmed this diagnosis. This case highlights the need to consider detailed chromosomal study for patients with delayed development and epilepsy. Furthermore, 1p36 deletion syndrome should be considered for patients presenting seizure and moderate-to-severe developmental delay, particularly if the patient exhibits dysmorphic features, short stature, and hypotonia.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.27 no.5
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• pp.478-481
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• 2005

Ossifying fibroma is a rare, benign, fibro-osseous tumor of mesenchymal origin. It develops mainly in the mandible, where it is usually slow growing and asymptomatic, whereas in the maxilla and paranasal sinus, it is more aggressive. The vast majority are located in the posterior region of the mandible; however, ossifying fibroma involving the maxillary sinus is uncommon. It may reach a very large size and cause facial asymmetry. It shows a female predilection, and most cases are seen in the third and fourth decades of life. Radiographically, ossifying fibroma is radiolucent or radiopaque depending on the amount of calcification. Histologically, the tumor consists of a cementum-like or bony mass. Ossifying fibroma shares many histopathologic features with fibrous dysplasia. Their radiographic features may help to separate these entities when pathological differentiation is uncertain. It is important to differentiate ossifying fibroma from fibrous dysplasia. The treatment of ossifying fibroma involves its complete removal using curettage, enucleation, excision, or en bloc resection. This case report presents an unusual case of ossifying fibroma of the maxillary sinus. We describe a Caldwell-Luc operation with a lateral window approach for excision of an ossifying fibroma of the maxillary sinus.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.15 no.3
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• pp.171-177
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• 1993

The prominent malar region has been recognized a sign of youth and beauty in caucasian who generally have a dolichocephalic and long face. But in the orients, especially Koreans who generally have a mesocephalic or brachycephalic face, it is considered an agressive, unesthetic facial appearance. So many patients require the shaving of prominent malar eminence and arch, and many methods of its reduction have been devised. For the exposure of malar complex, infraorbital skin incision, intraoral approach, preauricular approach, supraauricular scalp incision, and coronal approach have been used. And for the reduction of bony structure, direct shaving, contouring and repositioning of the malar complex after extirpation, and medially fracture of zygomatic arch have been used with its own merits. We performed the reduction malarplasty through intraoral approach. After two parallel oeteotomy at medial part of the zygomatic bone, the midsegment is removed. The posterior arch of zygoma was bended or green stick fractured. When more correction was required, the posterior arch was fractured medially through the step incision at skin. This method has a some advantages. Compared with the method for extirpation of malar complex, the infection rate is diminished, the resorption is small because of no free bone graft. And cheek drooping is prevented. Compared with the method of coronal approach, the surgical trauma is minimal. Now we report some cases of reduction malarplasty performed through intraoral approach and disscus the surgical technique and results.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.35 no.3
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• pp.189-194
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• 2013

Osteochondroma is a benign neoplasm, osseous projection surrounded with cartilage, 35.8% of benign osseous tumor, 8.5% of whole osseous tumor and usually arises from the skeletal bone. Osteochondroma is a cartilaginous derivation and relatively uncommon in the craniofacial bone. Osteochondroma of the mandible has slow growth rates which mainly affect women around forty years of age and it can appear through the coronoid process and mandibular condyle, especially in the medial half. Clinical finding associated with osteochondroma of condyle are primarily a palpable, painless temporomandibular area mass with facial asymmetry, malocclusion and midline deviations. Sometimes pain and dysfunction like trismus often accompany the anatomic derangement. Other features include malocclusion with open-bite on the affected side and cross-bite on the contralateral side. In this study, a 45-years old female patient exhibits pain on the left temporo-mandibular joint area and malocclusion due to loss of the molar region with osteochondroma on the top left of her mandibular condyle head. The patient is able to recover gradually from the symptom through treatments on manipulation, stabilization splint, arthroscopic lavage and surgical excision, thus, this is reported as a clinical case.

• The korean journal of orthodontics
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• v.50 no.2
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• pp.75-85
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• 2020

Objective: The aim of this study was to evaluate changes in the nasal soft tissues, including movements of landmarks, changes in linear distances, and volumetric changes, using three-dimensional (3D) stereophotogrammetry after microimplant-assisted rapid palatal expansion (MARPE) in adult patients. Methods: Facial data were scanned using a white light scanner before and after MARPE in 30 patients. In total, 7 mm of expansion was achieved over a 4-week expansion period. We determined 10 soft tissue landmarks using reverse engineering software and measured 3D vector changes at those points. In addition, we calculated the distances between points to determine changes in the width of the nasal soft tissues. The volumetric change in the nose was also measured. Results: All landmarks except pronasale and subnasale showed statistically significant movement on the x-axis. Pronasale, subnasale, alar right, and alar left showed significant movement on the y-axis, while all landmarks except subnasale showed significant movement on the z-axis. The alar base width, alar width, and alar curvature width increased by 1.214, 0.932, and 0.987 mm, respectively. The average volumetric change was 993.33 ㎣, and the amount of increase relative to the average initial volume was 2.96%. Conclusions: The majority of soft tissue landmarks around the nasal region show significant positional changes after MARPE in adults. The nose tends to widen and move forward and downward. The post-treatment nasal volume may also exhibit a significant increase relative to the initial volume. Clinicians should thoroughly explain the anticipated changes to patients before MARPE initiation.

• Clinical and Experimental Pediatrics
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• v.58 no.9
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• pp.354-357
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• 2015

Parry-Romberg syndrome (PRS) is a rare, acquired disorder characterized by progressive unilateral facial atrophy of the skin, soft tissue, muscles, and underlying bony structures that may be preceded by cutaneous induration. It is sometimes accompanied by ipsilateral brain lesions and neurological symptoms. Here we present the case of a 10-year-old girl with right-sided PRS and recurrent monoplegic ataxia of the left leg. At 4 years of age, she presented with localized scleroderma over the right parietal region of her scalp; her face gradually became asymmetric as her right cheek atrophied. Brain magnetic resonance imaging revealed hemiatrophy of the face and skull base, and T2-weighted images showed increased signal in the right hemipons and hemicerebellar peduncle. Magnetic resonance angiography findings were unremarkable. She was treated with oral prednisolone, and her recurrent gait ataxia diminished within 2 months of the follow-up period. To the best of our knowledge, this is only the second case of PRS presenting with an abnormal involvement of the ipsilateral hemipons.

• Archives of Plastic Surgery
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• v.39 no.1
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• pp.55-58
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• 2012

We experienced satisfactory outcomes by synchronously transplanting an artery and vein using an anterolateral thigh flap pedicle between the vascular pedicle and recipient vessel of a flap for scalp reconstruction. A 45-year-old man developed a subdural hemorrhage due to a fall injury. In this patient, the right temporal cranium was missing and the patient had $4{\times}3cm$ and $6{\times}5cm$ scalp defects. We planned a scalp reconstruction using a latissimus dorsi free flap. Intraoperatively, there was a severe injury to the right superficial temporal vessel because of previous neurosurgical operations. A 15 cm long pedicle defect was needed to reach the recipient facial vessels. For the vascular graft, the descending branch of the lateral circumflex femoral artery and two venae comitantes were harvested. The flap survived well and the skin graft was successful with no notable complications. When an interposition graft is needed in the reconstruction of the head and neck region for which mobility is mandatory to a greater extent, a sufficient length of graft from an anterolateral flap pedicle could easily be harvested. Thus, this could contribute to not only resolving the disadvantages of a venous graft but also to successfully performing a vascular anastomosis.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.37 no.3
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• pp.234-236
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• 2011

Traditional surgery to remove foreign bodies in the face carries a risk of postoperative morbidity with an injury to various anatomical structures, particularly the facial nerve and parotid duct and gland. Endoscopy can be a great aid in the removal of foreign bodies in the maxillofacial region. Surgical intervention using endoscope and/,or intraoperative images can be minimized, allowing the safe and precise removal of foreign bodies, and saving operating time. We report a case of the use of an endoscope and C-arm fluoroscopy guidance system to remove a very small foreign body.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.33 no.2
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• pp.81-93
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• 2007

Purpose: Adenoid cystic carcinoma (ACC) is a relatively rare tumor that arises in glandular tissues of the head and neck region and sometimes has a protracted clinical course with perineural invasion and delayed onset of distant lung metastasis. Treatment failure of salivary ACC is most often associated with perineural and hematogenous tumor spread. However, very little has been known about the cellular and molecular mechanisms of perineural invasion and hematogenous distant metastasis of parotid ACC. This study was designed to develop an orthotopic tumor model of parotid adenoid cystic carcinoma in athymic nude mice. Experimental Design: A melanoma cell line was injected into the parotid gland of athymic mice to determine whether such implantation was technically feasible. A parotid ACC cell line was then injected into the parotid gland or the subcutaneous tissue of athymic mice at various concentrations of tumor cells, and the mice were thereafter followed for development of tumor nodule. The tumors were examined histopathologically for perineural invasion or regional or distant lung metastasis. We used an oral squmous cell carcinoma cell line as control. Results: Implantation of tumor(melanoma) cell suspension into the parotid gland of nude mice was technically feasible and resulted in the formation of parotid tumors. A parotid ACC cell line, ACC3 showed no significantly higher tumorigenicity, but showed significantly higher lung metastatic potential in the parotid gland than in the subcutis. In contrast, mucosal squmous cell carcinoma cell line doesn’t show significantly higher lung metastatic potential in the parotid gland than in the subcutis. The ACC tumor established in the parotid gland seemed to demonstrate perineural invasion of facial nerve, needs further study. Conclusion: An orthotopic tumor model of salivary ACC in athymic nude mice was successfully developed that closely recapitulates the clinical situations of human salivary ACC. This model should facilitate the understanding of the cellular and molecular mechanisms of tumorigenisis and metastasis of salivary ACC and aid in the development of targeted molecular therapies of salivary ACC.

• The Journal of Advanced Prosthodontics
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• v.4 no.3
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• pp.153-157
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• 2012

PURPOSE. The study was conducted to compare the radiographic and clinical methods of measuring the horizontal condylar guidance (HCG) values. MATERIALS AND METHODS. The condylar guidance was measured using the radiographic (CT scan) and three clinical methods i.e. the wax protrusive records, Lucia jig record and intraoral central bearing device in 12 patients aged between 20-40 years irrespective of sex. The records were taken and transferred on the semi-adjustable articulator to record the HCG values. The CT scan was taken for 3D reconstruction of the mid facial region. Frankfort horizontal plane (FHP) and a line extending from the superior anterior most point on the glenoid fossa to the most convex point on the apex of articular eminence (AE) was marked on the CT scan. An angle between these two lines was measured on both right and left sides to obtain condylar inclination angle. Three interocclusal protrusive wax and jig records were taken and transferred to the semi adjustable articulator. Three readings were recorded on each side. Similarly the records were taken and transferred to the same articulator using the intra oral central bearing device to record the readings. RESULTS. The statistical analysis showed insignificant differences in the HCG values between the right and left sides [(P=.589 (CT), P=.928 (wax), P=.625 (jig), P=.886 (tracer)]. The clinical methods provided low Pearsons correlation values [(R = 0.423 (wax), R = 0.354 (jig), R = 0.265 (tracer)] for the right as well as the left sides when compared with the CT values. Among the clinical methods, jig and wax method showed strong level of association which is statistically significant while the intra-oral tracer showed weak association with the other two methods. CONCLUSION. The right and left HCG values were almost similar. The CT scan showed higher HCG values than the clinical methods and among the clinical methods, values obtained from all the methods were comparable.