• Journal of the korean academy of Pediatric Dentistry
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• v.36 no.4
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• pp.619-624
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• 2009

Pyknodysostosis(PKND) is a rare sclerosing bone disorder that has an autosomal recessive trait, also known as Toulouse-Lautrec syndrome. Deficiency of the cathepsin enzyme K in the osteoclasts of PKND patients results in continuous endosteal bone deposits without osteoclastic resorption or remodeling. This causes a generalized increase in sclerosis and fragility of bones. Osteomyelitis in the mandible and recurrent fracture of the long bones are characteristic complicatons of PKND. The patients present typical features of PKND, such as short stature under 150 cm, open cranial suture and fontanelle, club-shaped phalanges, and underdevelopment of midface. This is a case of a 7-year-old girl with PKND, who visited our clinic with the chief complaint of anterior Open-bite and generalized crowding. The patient had been diagnosed as PKND by an orthopedist and manifested characteristic clinical and radiographic features, such as open cranial suture and fontanelle, obtuse madibular gonial angle, frontal and occipital bossing, grooved palate, club-shaped phalanges, and short stature. Orthodontic treatment was not considered because patients with PKND show abnormal bone resorption and remodeling. Instead, removal of deciduous teeth near exfoliation and TFA were performed, and periodic check-up is planned to maintain good oral hygiene.

• The korean journal of orthodontics
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• v.30 no.6 s.83
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• pp.669-675
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• 2000

Excessive production of parathyroid hormone causes bony disorder such as periosteal bone resorption and bone pain due to excessive skeletal demineralization. A Class III facial deformity case with generalized root resorption presented bete was fumed out to be due to hyperparathyroidism. Clinical and cephalometric analysis revealed a straight skeletal profile with a retruded maxilla and a prognathic mandible. The x-ray findings demonstrated generalized root resorption of entire dentition to different degree. There also appeared osteoporosis like immature trabecular structure with the evidence of ground glass appearance. Serum test showed elevated 1evel of parathyroid hormone and growth hormone. Change of cranial growth by hyperparathyroidism can be dependent up(In a decreased bone apposition in viscerocranial growth site and abnormalities in cranial suture growth. It is possible to hypothesize that growth retardation of maxilla at least partially be accounted lot hyperparathyroidism. Therefore, regarding to the definite etiology of skeletal Class III and orthodontic treatment planning considering root resorption and osteoporosis, the early diagnosis for the hyperparathyroidism should be carefully carried by clinical and laboratory studies.

• Imaging Science in Dentistry
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• v.35 no.3
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• pp.133-139
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• 2005

Purpose To observe the histopathological changes in the periodontal tissues of mandibular molars in streptozotocin-induced diabetic rats after irradiation. Materials and Methods : The male Sprague-Dawley rats weighing approximately 250 gm were divided into four groups: control, diabetes, irradiation, and diabetes- irradiation groups. Diabetes mellitus was induced in the rats by injecting streptozotocin. Rats in the control and irradiation groups were injected with citrate buffer only After 5days, the head and neck region of the rats in irradiation and diabetes-irradiation groups were irradiated with a single absorbed dose of 10Gy. All the rats were sacrificed at 3, 7, 14, 21, and 28 days after irradiation. The specimen including the mandibular molars were sectioned and observed using a histopathological method. Results In the diabetes group, osteoclastic activity was observed in the alveolar bone and the root throughout the period of experiment. Also, osteoblastic and fibroblastic activities were markedly decreased. In the irradiation group, the osteoclasts were observed in the alveolar bone and the dilated capillaries were increased in the early experimental phases. However, vigorous osteoblastic activity was noted in the late experimental phases. In the diabetes-irradiation group, osteoblastic activity in the alveolar bone and the root was observed in the early experimental phases. However, there were no resorption and osteoblastic activity in the alveolar bone and the root in the late experimental phases, and obvious atrophic change of fibrous tissues was noted. Conclusion : This experiment suggests that osteoblastic activity was caused by irradiation in the late experimental phases, but atrophic change of the periodontal ligament tissues was induced after irradiation in diabetic state.

• The korean journal of orthodontics
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• v.28 no.4 s.69
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• pp.499-515
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• 1998

The purpose of this study was to examine the associations of head posture the position of the tongue or the hyoid bone to craniofacial structure. Cephalograms taken in Natural head position(NHP) of 90 dental students (50 in male, 40 in female, 20 to 30 years in age) were traced and measured using the extracranial true horizontal and vertical lines. The obtained results were as follows; 1. There was no sex difference in head posture, but the hyoid bone was placed anteroinferiorly in male more than in female and anteroinferior inclination of the hyoid bone showed greatly in male. 2. The more inclined was the cervical column, the less prognathic was the face in natural head posture, and the larger cervical curvature, the more vertical pattern of the face. 3. The less small showed craniocervical angulation, the more anteriorly placed was the hyoid bone to the cranial base, and there was no significantly association between craniocervical angulation and the vertical position of the hyoid bone. 4. The more prognathic was the mandible, the more anteriorly placed was the hyoid bone, and there was slightly association between the craniofacial morphology and the vertical position of the hyoid bone.

• Journal of Periodontal and Implant Science
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• v.34 no.3
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• pp.543-549
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• 2004

Chitosan has been widely researched as bone substitution materials and membranes in orthopedic/periodontal applications. Chitosan nanofiber membrane was fabricated by chitosan nanofiber using electrospinning technique. The structure of the membrane is nonwoven, three-dimensional, porous, and nanoscale fiber-based matrix. The aim of this study was to evaluate the biocompatibility of chitosan nanofiber membrane and to evaluate its capacity of bone regeneration in rabbit calvarial defect. Ten mm diameter round cranial defects were made and covered by 2 kinds of membranes (Gore-Tex membrane, chitosan nanofiber membrane) in rabbits. Animals were sacrificed at 4 weeks after surgery. Decalcified specimens were prepared and observed by microscope. Chitosan nanofiber membrane maintained its shape and space at 4 weeks. No inflammatory cells were seen on the surface of the membrane. In calvarial defects, new bone bridges were formed at all defect areas and fused to original old bone. No distortion and resorption was observed in the grafted chitosan nanofiber membrane. However bone bridge formation and new bone formation at the center of the defect could not be seen in Gore-Tex membranes. It is concluded that the novel membrane made of chitosan nanofiber by electrospinning technique may be used as a possible tool for guided bone regeneration.

• Journal of Korean Neurosurgical Society
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• v.30 no.sup2
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• pp.216-220
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• 2001

Objective : Cranioplasty is required to protect underlying brain, to correct major aesthetic deformities, or both. The ideal material for this purpose is autogenous bone. When this is not available, alloplastic or artificial materials may be used. In this study authors compared the infection rate according to the cranioplasty materials(the frozen autologous bone vs. bone cement), and duration of the skull defect. Materials : Between May 1994 and December 1999, 111 patients with skull defect treated with cranioplasty(82 cases of frozen autologous bone and 29 cases of artificial bone material) were included in this study. There were 77 males and 34 females with a mean age of 41.4 years(range 1-85 years). 57 patients had head trauma and 54 had non-traumatic insults. According to the duration of skull defect, there were 28 cases under 1 month, 33 cases of 1-2 months, 15 cases of 2-3 months, 20 cases of 3-6 months and 15 cases over 6 months of duration. Results : Overall infection rate was 9.9%. In cases with frozen autologous bone and artificial bone material, the infection rate was 8.5% and 13.7%, respectively. The infection rate according to the duration of skull defect was 3.6%(among 28 cases) under 1 month of age, while those were 12%(4 among 33 cases) at 1-2 months, 20%(3 among 15 cases) at 2-3 months, 5%(1 among 20 cases) at 3-6 months and 13%(2 among 15 cases) over 6 months. Accoring to the underlying disease, the infection rate in traumatic cases was 12%(7 among 57 cases) and that in non-traumatic one was 3.7%(2 among 54 cases). Conclusion : From this study, it appears that skull defect should be repaired as soon as possible, because early cranioplasty can lower the infection rate. And surgeons could save the patients' cranial bone as possible as they can because autologous bone is not only cost effective in cosmatic purpose but lower the infection rate.

• Korean Journal of Head & Neck Oncology
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• v.23 no.1
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• pp.41-45
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• 2007

A 60 years old female patient presented with $8{\times}6\;cm$ sized painless oval mass in the left parietal region. She had left lobectomy of thyroid gland 10 years ago. Cranial CT, MRI, FGD PET-CT showed a solid mass which invaded left parietal bone. After embolization, craniectomy with tumor excision was performed. Histological examination revealed metastatic follicular cancer originated thyroid gland, with vascular and dura invasion. Postoperatively, neck CT showed right thyroid multiple nodules and right level III multiple lymph node enlargement. Thyroid function test was normal, but level of thyroglobulin was high (72ng/ml). So she had right lobectomy of thyroid gland with lymph node dissection under a diagnosis of follicular carcinoma. But histological examination revealed adenomatous hyperplasia and not lymph node metastasis. After operation, she received radioiodine therapy of 150mCi and then the level of thyroglobulin normalized (8.4ng/ml). The patient is under follow-up since she had operation 4 months ago.

• Archives of Plastic Surgery
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• v.35 no.3
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• pp.316-320
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• 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.

• Korean Journal of Veterinary Research
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• v.35 no.4
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• pp.643-649
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• 1995

An abnormal Korean native cow of six years old with extra legs was observed macroscopically and radiographically. The results were summarized as follows; 1. External features included two normal forelimbs and hindlimbs, and two extra forelimbs which were underdeveloped. The extra forelimbs of different length attached laterally to the cranial regions of neck on right scapula. 2. The large extra forelimb had scapular meromelia. It consisted of underdeveloped humerus, antebrachial skeleton, several carpal bones, metacapal bone(MC3+4) and two digitis with each pairs of hoof and a rudimentary hoof of dewclaw. 3. The small extra forelimb had scapular and antebrachial meromelia. It consisted of underdeveloped metacarpal and carpal bone, severely deformed humerus, fused carpal bones, and two digitis with two hooves, and a rudimentary hoof of dewclaw. 4. The dermoid cyst was observed between the large and small extra forelimbs.

• Archives of Craniofacial Surgery
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• v.10 no.1
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• pp.37-39
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• 2009

Purpose: Langerhans cell histiocytosis is a heterogenous group of Langerhans cell proliferative disorders and includes eosinophilic granuloma, Letterer-Siwe diseases, and Hand-Schuller Christian disease. We report a case of eosinophilic granuloma on frontal area. Methods: A 17-year-old male presented with swelling and tenderness on Lt. frontal and periorbital area. CT and MRI showed a $33{\times}25mm$ sized mass that involved Lt. frontal calvarium, frontotemporal meninges, and orbital roof. Results: Total excision of the mass and adjacent soft tissue, calvarium, and orbital roof was performed. Orbital roof defect was reconstructed with absorbable plate and calvarial defect was done with outer cortex of temporal bone flap. The histology revealed proliferation of histiocytes and eosinophils. Immunologically, these histiocytic cells expressed S-100 protein and CD1a. The patient is currently taking conservative treatment. Conclusion: The severity of these disease and their prognosis and treatments are various. For unifocal cranial Langerhans cell histiocytosis, complete excision is the treatment of choice. We report this case with review of literature.