• Title/Summary/Keyword: Fibrous Dysplasia

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FIBROUS DYSPLASIA CAUSING FACIAL ASYMMETRY : A CASE REPORT (안면비대칭을 유발한 섬유성이형성증 : 증례보고)

  • Park, Min-Kyoung;Jeong, Younwook;Lee, Hyo-Seol;Song, Je-Seon;Choi, Byung-Jai;Lee, Jae-Ho
    • The Journal of Korea Assosiation for Disability and Oral Health
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    • v.10 no.1
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    • pp.22-25
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    • 2014
  • Fibrous dysplasia(FD) is a benign fibro-ossifying disease in which fibrous tissue replaces normal bone and marrow. Craniofacial bones, including the maxilla and mandible, are commonly involved. A 7 year-old girl visited the clinic with a chief complaint of gingival swelling around the lower left primary molar. Mild bulging of the lower left periodontal tissue was observed. Not only the mandible, but also the maxilla, zygoma, sphenoid, and temporal bones were affected by FD. Permanent tooth germs were involved in the lesions and facial asymmetry was caused by lower left bone expansion. She was scheduled for a follow-up visit at the department of pediatric dentistry and oromaxillofacial surgery.

CHARACTERISTICS OF FIBROUS DYSPLASIA DERIVED CELLS (섬유성이형성증 유래세포의 특성연구)

  • Lee, Chan-Hee;Han, Ihn;Seo, Byoung-Moo
    • Journal of the Korean Association of Oral and Maxillofacial Surgeons
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    • v.35 no.5
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    • pp.304-309
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    • 2009
  • Purpose: Fibrous dysplasia (FD) is a fibro-osseous disease associated with activating missense mutations of the gene encoding the $\alpha$-subunit of stimulatory G protein. FD may affect a single bone (called monostotic form) or multiple bones (called polyostotic form). The extent of lesions reflects the onset time of mutation. In this study, cells from monostotic FD in maxilla of a patient were isolated and cultured in vitro for characterization. Materials and Methods: The single cells were released from FD lesion which was surgical specimen from 15 years-old boy. These isolated cells were cultured in vitro and tested their proliferation activity with MTT assay. In osteogenic media, these cells underwent differentiation process comparing with its normal counterpart i.e. bone marrow stromal cells. The proliferated FD cells were detached and transplanted into the dordsal pocket of nude mouse and harvested in 6 weeks and 12 weeks. Results and Summary: FD cells have an increased proliferation rate and poor differentiation. As a result, cells isolated from FD lesion decreased differentiation into osteoblast and increased proliferation capacity. MTT assay presented that proliferation rate of FD cells were higher than control. However, the mineral induction capacity of FD was lesser than that of control. Monostotic FD cells make fewer amounts of bone ossicles and most of them are woven bone rather than lamellar bone in vivo transplantation. In transplanted FD cells, hematopoietic marrow were not seen in the marrow space and filled with the organized fibrous tissue. Therefore, they were recapitulated to the original histological features of FD lesion. Collectively, these results indicated that the FD cells were shown that the increased proliferation and decreased differentiation potential. These in vitro and in vivo system can be useful to test FD cell's fate and possible.

Psammomatoid Juvenile Ossifying Fibroma of the Maxilla Misdiagnosed as Fibrous Dysplasia: A Clinicopathologic Case Report

  • Jong-Ho Kim;Jiwon Kang;Seong-ik Kim;Byung Jun Kim
    • Archives of Plastic Surgery
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    • v.50 no.1
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    • pp.49-53
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    • 2023
  • Juvenile ossifying fibroma (JOF) is a variant of the ossifying fibroma and includes two histopathological subtypes: trabecular and psammomatoid. Psammomatoid JOF (PJOF) in craniofacial structures should be distinguished from other fibro-osseous lesions, such as fibrous dysplasia (FD), considering the difference in the treatment protocols. Here, we present a rare case of PJOF that was initially misdiagnosed as a case of FD and emphasize the importance of considering JOF in the differential diagnosis of patients with craniofacial fibro-osseous lesions. A 4-year-old boy demonstrated progressive enlargement of the zygomaticomaxillary area on his left side for the last 6 months. The patient was diagnosed as a case of FD based on the clinical features and radiographic findings, and was operated considering the rapid progression. To achieve facial symmetry, contouring of the zygomatic bone and arch was performed. However, the patient demonstrated rapid enlargement at the 3-month postoperative follow-up. The decision was made to surgically remove the tumor due to visual field impairment. Intraoperatively, a rubbery mass, which was separated from the surrounding cortical bone, was identified and excised. The lesion was confirmed as PJOF by histopathological examination. The possibility of PJOF should not be ruled out in the differential diagnosis of patients with fibrous-osseous lesions. In the event of suspected PJOF, accurate diagnosis should be made through definitive biopsy.

The Diagnostic importance of clinical and radiologic features of the Multiple Cemento-osseous dysplasia (다발성 백악질공이형성증 조직병리검사시 임상, 방사선양상의 중요성)

  • Han Mi-Ra;Kim Young-Hee;Kang Byung-Cheol
    • Journal of Korean Academy of Oral and Maxillofacial Radiology
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    • v.28 no.1
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    • pp.299-309
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    • 1998
  • This case was diagnosed as multiple cementoosseous dysplasia on the basis of clinical & radiological features but was diagnosed as ossifying fibroma on the basis of histopathological feature. The histopathologic features of the multiple cementoosseous dysplasia and cementoossifying fibroma have common features of cementum, fibrous network and bone. Multiple cementoosseous dysplasia is reactive lesion and shows restricted lesion size, occurred on anterior and posterior tooth of the mandible and needs no treatement except periodic follow up. But Cementoossifying fibroma is the true neoplasm and grows continuously and needs surgical removal. The final diagnosis of the multiple cementoosseous dysplasia requires good correlation of the clinical, histopathological, and radiological features.

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A CASE OF DELAYED ERUPTION IN A CHILD WITH MONOSTOTIC FIBROUS DYSPLASIA (단골성 섬유성이형성증 환아에서 나타난 맹출지연)

  • Min, Soo-Young;Lee, Jae-Ho;Kim, Seong-Oh;Choi, Byung-Jae;Choi, Hyung-Jun;Kim, Seung-Hye;Song, Je-Seon
    • Journal of the korean academy of Pediatric Dentistry
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    • v.38 no.3
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    • pp.270-275
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    • 2011
  • Fibrous dysplasia is a developmental tumor-like condition that is characterized by replacement of normal bone by an excessive proliferation of cellular fibrous connective tissue intermixed with irregular bony trabeculae. Craniofacial lesions may cause facial pain, headache, cranial asymmetry, facial deformity, tooth displacement and visual or auditory impairment. In this case, a 2-year-9-month old boy who was diagnosed as the fibrous dysplasia showed delayed eruption on affected area. Teeth of left lateral dentition group have erupted completely but teeth of right lateral dentition group are erupting slowly. Eruption and maturation of affected teeth are in progress, so continuous observation is required presently. If the eruption state stops, surgical opening or forced eruption of the impacted teeth will be considered.

A Case of McCune-Albright Syndrome with Vitamin D Resistant Rickets (비타민 D 저항성 구루병을 동반한 McCune-Albright 증후군 1례)

  • Kang Hee;Choe Jeong-Hoon;Hong Young-Sook;Lee Joo-Won;Kim Soon-Kyum;Yoo Kee-Hwan
    • Childhood Kidney Diseases
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    • v.3 no.2
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    • pp.237-240
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    • 1999
  • The presence of polyostotic fibrous dysplasia of bone, hyperpigmented skin macules, and precocious sexual development in children is known as the McCune-Albright syndrome. In addition to the described in McCune-Albright syndrome, other endocrinopathies have been reported including hyperthyroidism, acromegaly, Cushing syndrome and vitamin D resistant rickets. The case describes a 6-year-old boy showing bony deformities due to polyostotic fibrous dysplasia, hyperpigmented skin macules, hyperthyroidism and vitamin D resistant rickets. The purpose of this report is to describe a patient of McCune-Albright syndrome with vitamin D resistant rickets which is very rare.

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TREATMENT OF POLYOSTOTIC FIBROUS DYSPLASIA DEVELOPED IN LEFT CRANIOFACIAL BONES:A CASE REPORT (좌측 두개 안면부에 발생한 다골성 섬유성 골 이형성증의 치험례)

  • Kim, Il-Kyu;Lee, Seong-Jun;Ha, Soo-Yong;Chu, Young-Chae
    • Maxillofacial Plastic and Reconstructive Surgery
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    • v.12 no.2
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    • pp.95-101
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    • 1990
  • This is a case report of polyostotic fibrous dysplasia developed in the craniofacial region of 21 year old male patient, who had complained the buccolingual expansion of left mandibular body area, malocclusion and facial asymmetry. We could achieve satisfactory results by radical resection of the relatively well defined small lesion of mandible and by cosmetic bone shaving procedure on the widely dispersed and poorly defined lesions of cranium. But the persistent growth and recurrence of the lesions may produce loss of hearing, visual difficulties, facial paralysis and anosmia, and as it is a polyostotic type occured in the craniofacial region of male patient, the possibility of malignant degeneration should not be excluded completely and periodic recall and check up will be necessary.

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Fibrous Dysplasia of the Temporal Bone (측두골섬유성리형성증)

  • 김종선;우훈영;백만기
    • Proceedings of the KOR-BRONCHOESO Conference
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    • 1979.05a
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    • pp.5.1-5
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    • 1979
  • Fibrous dysplasia of the temporal bone is a extremely rare condition in otological field with a total of 11 cases in the English literature so far. Authors experienced a case in 18 year old boy with complete obliteration of the external auditory canal and long standing keratosis obturance. The purpose of this paper is to discuss principles and methods of canalplasty and meatoplasty with literature review and presentation of authors case.

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