• 한국임상수의학회지
• /
• 제34권2호
• /
• pp.108-111
• /
• 2017

A 4-year-old, weighing 7.6 kg, castrated male, Pug presented with ingestion of gastric cavity foreign body. Physical examination revealed panting, retching and hyper-salivation. Blood chemistry and complete blood cell count were normal, but hypophosphatemia was observed. An abdominal radiograph revealed the foreign body (FB), round shape and 2 cm length, at the pyloric region of stomach. A thoracic radiograph revealed an incidental metal FB, 3.5 cm length, at the cranial portion of the diaphragm. An upper gastrointestinal endoscopy was performed to remove the FB in the stomach and then a peach-pit was removed. However the metal FB was not found in the esophagus therefore a lateral thoracotomy was performed. A right lateral thoracotomy through the $7^{th}$ intercostal space was accomplished to expose the right caudal lung lobe. After open the thoracic cavity, foreign body was not observed by gross evaluation and caudal lung lobe was attached to the diaphragm. The FB was identified inside the lung lobe and surrounded by granulation tissue. The metal FB (sewing needle) was removed with blunt dissection and incised lung lobe was sutured using absorbable suture material PDS 4-0 with interrupted suture. A thoracotomy tube was inserted into the thoracic cavity during surgery. Patient's respiration became stable after surgery. A chest tube was removed 3 days after surgery. No complications were noted and the dog was discharged 4 days after surgery. In small animal, foreign body ingestion is a common reason for emergency. After ingestion of the FB, perforation through the esophagus and migration to inside the lung lobe is not common in small animals. In this case, thoracic metal FB was identified incidentally and removal of a thoracic FB with thoracotomy was performed successfully.

• Archives of Plastic Surgery
• /
• 제38권1호
• /
• pp.77-80
• /
• 2011

Purpose: We report a patient with DiGeorge syndrome who was later diagnosed as mild metopic synostosis and received anterior 2/3 calvarial remodeling. Methods: A 16-month-old boy, who underwent palatoplasty for cleft palate at Chungnam National University Hospital when he was 12 months old of age, visited St. Mary's Hospital for known DiGeorge syndrome with craniosynostosis. He had growth retardation and was also diagnosed with hydronephrosis and thymic agenesis. His chromosomal study showed microdeletion of 22q11.2. On physical examination, there were parieto-occipital protrusion and bifrontotemporal narrowing. The facial bone computed tomography showed premature closure of metopic suture, orbital harlequin sign and decreased anterior cranial volume. The interorbital distance was decreased (17 mm) and the cephalic index was 93%. Results: After the correction of metopic synostosis by anterior 2/3 calvarial remodeling, the anterior cranial volume expanded with increased interorbital distance and decreased cephalic index. Fever and pancytopenia were noted at 1 month after the operation, and he was diagnosed as hemophagocytic lymphohistiocytosis by bone marrow study. He however, recovered after pediatric treatment. There was no other complication during the 12 month follow up period. Conclusion: This case presents with a rare combination of DiGeorge syndrome and metopic synostosis. When a child is diagnosed with DiGeorge syndrome soon after the birth, clinicians should keep in mind the possibility of an accompanying craniosynostosis. Other possible comorbidities should also be evaluated before the correction of craniosynostosis in patients as DiGeorge syndrome. In addition, postoperative management requires a thorough follow up by a multidisciplinary team of plastic surgeons, neurosurgeons, ophthalmologists and pediatricians.

• 대한소아치과학회지
• /
• 제30권4호
• /
• pp.643-653
• /
• 2003

막내골화와 연골내골화 등의 정상적인 골격 성장은 섬유아세포 성장인자 (FGF) 와 이들 수용체들 (FGFR) 에 의한 신호 전달체계에 의해 조절된다. 또한 전사조절인자인 Runx2 ($Cbfa1/Pebp2{\alpha}A/AML3$) 는 골아세포분화 뿐만 아니라 골형성에도 필수적인 유전자로 알려져 있다. FGF signaling이 mouse의 두개관과 하악에서의 연골 형성에 어떤 영향을 미치고 있으며, 이 과정에서 Runx2의 연관성을 알아보고자 in vivo 및 in vitro 실험을 시행하여 다음과 같은 결과를 얻었다. 두개관과 하악을 Alcian blue 염색한 결과 시상두개봉합부 연골은 태생 16일부터, Meckel's 연골은 태생11일부터 형성되기 시작하였다. 이들 연골세포들의 성상을 알아보기위한 in situ hybridization 결과 시상두개봉합부 연골 및 Meckel's 연골 모두에서 type II collagen은 발현되었으나, Type X collagen은 발현되지 않았다. Runx2 mRNA는 시상두개봉합부 연골과 Meckel's 연골 모두에서 발현되지 않았지만, 이들 연골들의 가장자리를 둘러싸고 있는 독특한 발현양상을 나타내었다. 두개봉합부에서의 FGF2 protein의 국소적 적용은 두개봉합부 하방의 연골형성을 억제하였다. 또한 하악의 Meckel's 연골 발생 부위에 FGF2 protein의 국소적 적용 역시 Meckel's 연골의 형성을 억제하였다. FGF2 protein은 시상두개봉합부상의 bead 주위로 Runx2의 발현을 유도하였다. 이 결과들을 종합해볼 때, FGF signaling은 골아세포와 연골아세포가 공존하고 있는 조직에서의 연골 형성을 억제하고 있음을 시사해 주고 있으며, 이 과정에서 FGF signaling은 부분적으로 Runx2 유전자의 발현을 조절하므로써 연골세포의 증식과 분화에 관여할 것으로 사료된다.

• Archives of Plastic Surgery
• /
• 제35권3호
• /
• pp.316-320
• /
• 2008

Purpose: Pachydermoperiostosis is a rare hereditary disease characterized by finger clubbing, periosteal reaction, and pachydermia. The underlying pathogenic mechanism of this disease remains unclear. This disease is known to be associated with a variety of diseases such as cranial suture defect, bone marrow failure, hypertrophic gastropathy, Crohn's disease, and female escuchen. Methods: A 50-year-old male had digital clubbing of both hands, coarse hypertrophic skin changes of face, progressive thickening and furrowing on the scalp(cutis verticis gyrata), persistent pain in the limbs and joints. Other cutaneous features include moderate blepharoptosis, pole-like lower legs and feet. Results: We performed surgical excision for hypertrophic skin change of scalp because of frequent eczematous skin change, severe itching sensation and cosmetic problem. Diagnosis is confirmed by bony proliferative periosteal reaction, pathologic findings, and characteristic clinical findings. Conclusion: Pachydermoperiostosis is manifested by finger clubbing, and hypertrophic skin changes causing coarse facial features with thickening and periosteal bone formation. We experienced a case of pachydermoperiostosis. Brief review of related literature is given.

• Journal of Korean Neurosurgical Society
• /
• 제59권3호
• /
• pp.187-191
• /
• 2016

Craniosynostosis is defined as the premature fusion of one or more of the cranial sutures. It leads not only to secondary distortion of skull shape but to various complications including neurologic, ophthalmic and respiratory dysfunction. Craniosynostosis is very heterogeneous in terms of its causes, presentation, and management. Both environmental factors and genetic factors are associated with development of craniosynostosis. Nonsyndromic craniosynostosis accounts for more than 70% of all cases. Syndromic craniosynostosis with a certain genetic cause is more likely to involve multiple sutures or bilateral coronal sutures. FGFR2, FGFR3, FGFR1, TWIST1 and EFNB1 genes are major causative genes of genetic syndromes associated with craniosynostosis. Although most of syndromic craniosynostosis show autosomal dominant inheritance, approximately half of patients are de novo cases. Apert syndrome, Pfeiffer syndrome, Crouzon syndrome, and Antley-Bixler syndrome are related to mutations in FGFR family (especially in FGFR2), and mutations in FGFRs can be overlapped between different syndromes. Saethre-Chotzen syndrome, Muenke syndrome, and craniofrontonasal syndrome are representative disorders showing isolated coronal suture involvement. Compared to the other types of craniosynostosis, single gene mutations can be more frequently detected, in one-third of coronal synostosis patients. Molecular diagnosis can be helpful to provide adequate genetic counseling and guidance for patients with syndromic craniosynostosis.

• 대한치과교정학회지
• /
• 제30권6호
• /
• pp.669-675
• /
• 2000

부갑상선 호르몬이 과도하게 분비되는 경우 전반적인 골의 탈석회화에 의해 골막성 골흡수 및 골의 동통 등의 증상이 발현될 수 있다. 본고에서 소개된 환자의 경우 광범위한 치근 흡수를 동반한 부정 교합 환자로, 임상 및 두부 방사선 계측사진 분석에서 상악 열성장과 하악 과성장이 동반된 골격성 III급 부정 교합으로 진단되 었으며, 병리검사결과 부갑상선 호르몬 및 성장 호르몬의 수치가 증가된 것으로 보아 부갑상선 기능항진증으로 판단되었다. 방사선 사진 분석 결과 광범위한 치근의 흡수를 관찰할 수 있었으며 골다공증과 유사하게 골소주 형태가 매우 성기면서 ground glass 양상을 보였다. 이와 같이 부갑상선 기능항진증에 의해 골질뿐만 아니라 치근이 영향을 받은 것은 매우 드문 경우로 생각된다. 또한 부갑상선 기능항진증에 의해 내장두개의 골침착이 감소될 수 있다는 동물실험 결과를 볼 때 III급 부정교합의 원인이 상악 열성장인 점과 연관하여 매우 흥미로운 점으로 지적된다. 따라서 부갑상선 기능항진증의 다양한 증상 및 양태에 대한 조기진단 및 이에 대한 지식은 교정진단 및 치료에 중요한 역할을 할 수 있다고 생각된다.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
• /
• 제32권4호
• /
• pp.360-373
• /
• 2006

The purpose of this research was to investigate whether pulsed electromagnetic field (PEMF) stimulation applied to the rabbit cranial defects grafted with ${\beta}$-tricalcium phosphate (${\beta}$-TCP) could affect the new bone formation. With 16 New Zealand white rabbits under the same condition, bilateral calvarial bone defects were formed around the sagittal suture line. The defect on the left side was grafted with ${\beta}$-TCP, while on the right side was grafted by harvested autogenous bone. PEMF was applied to 8 rabbits for 8 hours per day. The bony specimen were divided into 3 groups, the group 1 was autogenous bone grafted specimen, the group 2 was ${\beta}$-TCP grafted with PEMF, and the group 3 was ${\beta}$-TCP grafted without PEMF. We investigated the bone regeneration & growth factor expression at 2, 4, 6, and 8 weeks. As a result, BMP 2 was expressed in the group 1 from 2 weeks, the group 2 from 4 weeks, and the group 3 from 6 weeks. BMP 4 was expressed in the group 1 from 2 weeks, in the group 2 and the group 3 from 4 weeks. 4. There was no significant difference in expression pattern of BMP 7, PDGF, VEGF, and TGF-${\beta}$1 during grafted bone regeneration in group 1, 2, and 3. According to our results, PEMF stimulation could be effective on the new bome formation in animal study, and have a feasibility of clinical use.

• 대한치과교정학회지
• /
• 제33권6호
• /
• pp.485-497
• /
• 2003

악안면 구조의 형태와 기능은 대개 유전자 정보에 의해 결정된다. 분자생물학의 발달로 인해 정상 성장과 형태 형성에 중요한 유전자에 대한 정보가 밝혀지고 있고 이는 현대 두개안면 생물학의 근간이 되고 있다. 밝혀진 사실들 중 주목할만한 것은 섬유아세포 성장인자2 (FGFR2)에 서 의 특이한 돌연변이 가 어 퍼트 증후군 (Apert syndrome) 의 발생과 관련이 있다는 것이다. 어퍼트 증후군은 두개 관상봉합의 조기 유합과 사지의 기형으로 특징지워진다. 그 중 특히 두개골 유합증의 병인과 형성기전을 연구하기 위해 본 연구에서 유전자 변환기법을 시도하여 어퍼트 증후군의 유발인자로 알려진 FGFR2에 서 의 단일 아미노산 치 환 돌연변이를 재연한 인위 유전자구조물을 제작하고 이를 미 세주입법으로 쥐의수정란에 삽입하여 형질변환 쥐를 제작하였다. 본 연구에서는 전체 조직이 아닌 골조직에서 특이하게 활성화되는 전사촉진자(promoter, 제 I형 교원질 유전자의 전사촉진자)를 이용하여 골조직에서만 돌연변이 유전자의 발현을 재현함으로써 이 시도가 쥐에서 두개골유합증을 유발하는지 검증하고자 하였다. 초기 표현형 분석을 통해 어퍼트 환자에서 기대되는 두개골 유합증을 확인하였다. 또한 본 연구에서 삽입된 변환유전자가 원활히 돌연변이 단백질을 생산하고 기능의 증가를 보임을 확인하였다. 이러한 동물 모델을 이용함으로써 이제 정상적 혹은 비정상적 두개골 및 봉합 발육에서의 FGFR2의 역할을 연구하는 것이 가능하리라 사료된다

• 대한두개안면성형외과학회지
• /
• 제9권2호
• /
• pp.85-89
• /
• 2008

Purpose: Unicoronal synostosis is the craniofacial anomaly caused by premature fusion of unilateral coronal suture. Ipsilateral flattening of the frontal and parietal bones, temporal retrusion with elevation and recession of the supraorbital rim are main clinical features. Compensatory contralateral frontal bossing and deviation of the nasal root and/or chin can also occur. There is a controversy about techniques for surgical correction, however, bilateral approach technique is more effective for correction of deformity. Methods: A 4-year-old patient with unicoronal synostosis had undergone unilateral suturectomy at 28-month-old but fronto-facial deformity had remained and aggravated as she grew older. She had both fronto-facial and endocranial asymmetry. We performed coronal cranial approach and fully exposed affected cranium including supraorbital rim. Anterior 2/3 calvarial reconstruction with bilateral frontal bone osteotomy and fronto-orbital bandeau advancement was performed. Results: Fronto-facial symmetry including fronto-orbital contour, nasal devation was improved. Endocranial twisting was also improved from $158^{\circ}$ to $162^{\circ}$ in CSO(crista gallisella turcica-opisthion) degree. There was no postoperative complications and no need for revision, and facial asymmetry improved at the period of 2 years of follow-up. Conclusion: Bilateral approach with fronto-orbital bandeau remodeling in surgery of unicoronal synostosis looked superior to unilateral approach in achieving better symmetry and preventing recurrence of asymmetry. Remodeling surgery should be tried in patients even at an older age to correct fronto-facial asymmetry.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
• /
• 제28권4호
• /
• pp.264-273
• /
• 2002

Pulsed electromagnetic field (PEMF) was used first to induce osteogenesis in 1974. The appliance which was consisted of the Helmholtz coil configuration have used to osteogensis. The objective of this study was to determine whether PEMF, a frequency of 100 Hz and magnetic field strength of 38 gauss applied to the calvarial defect in rabbit, could affect the induction of osteogenesis and the healing of the graft bone. This field should not produce excitation of nerve or muscle and heating the tissue. To evaluate the effect of PEMF on osteogenesis, 16 rabbit under the same condition was divided into 8 experimental groups and 8 control groups. 10 mm calvarial bone defects were formed around sagittal suture. The defect of left side was left without graft while the defect of right side was grafted by bone harvested from left side. A pulsed electromagnetic field was applied for 8 hours per day. Each group was sacrificed after 1 week, 2 weeks, 4 weeks, 8 weeks. Microscopic specimens were obtained from the calvarial bone defects and surrounding tissue using Hematoxylin-Eosin staining method. The results were as follows. 1. In the group which pulsed electromagnetic field was applied, new bone formation filled up the defect was observed after 4 and 8 weeks effectively. 2. There are no difference in the healing period for the fusion between the bone and graft bone. According to the result, the PEMF with 38 Gauss, 100 Hz was very effective in the healing of bone defect and new bone formation. So The PEMF will be useful in clinical aspect for oseteogenesis.