• Title/Summary/Keyword: Monostotic

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Monostotic Fibrous Dysplasia in the Metacarpal Bone: A Case Report (중수골에 발생한 단골성 섬유성 이형성증: 1예 보고)

  • Cho, Han-Il;Park, Sung-Hoon;Juhng, Seon-Kwan
    • Investigative Magnetic Resonance Imaging
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    • v.13 no.2
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    • pp.203-206
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    • 2009
  • Fibrous dysplasia is a slowly progressive, benign disorder characterized by fibrous tissue replacement of skeleton and may affect solitary or multiple bones. Monostotic fibrous dysplasia mainly occurrs in the rib, femur and tibia, however, rarely in the hand. We report a case of monostotic fibrous dysplasia confined to the 2nd metacarpal bone with findings of plain radiographs and MR imaging.

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Monostotic Fibrous Dysplasia in the Spine - A Case Report - (척추에 발생한 단골성 섬유 이형성증 - 증례 보고-)

  • Yang, Jun-Young;Lee, June-Kyu;Lee, Jun-Ho;Yang, Jae-Hoon
    • The Journal of the Korean bone and joint tumor society
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    • v.11 no.2
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    • pp.188-193
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    • 2005
  • Fibrous dysplasia is a condition characterized by the replacement of the medullary component of predominantly long bone with fibrous tissues. The monostotic form of the fibrous dysplasia occurs more frequent than the polyostotic form. Only the twenty three previous cases of vertebral involvement in monostotic fibrous dysplasia have been reported. Authors experienced a case of monostotic fibrous dysplasia of the third lumbar vertebra, so we report this case. Report details the diagnosis and treatment of a 34 years old man with back pain from monostotic fibrous dysplasia of the third lumbar vertebra. We discuss the experience in the consideration of previous report to recommend the optimal management of this disease.

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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.

Fibrous dysplasia of the maxilla in an elderly female: Case report on a 14-year quiescent phase

  • Lee, Byung-Do;Lee, Wan;Park, Yong-Chan;Kim, Myoung-Hyoun;Choi, Moon-Ki;Yoon, Jung-Hoon
    • Imaging Science in Dentistry
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    • v.46 no.4
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    • pp.259-265
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    • 2016
  • Fibrous dysplasia (FD) is an uncommon skeletal disorder in which normal bone is replaced by abnormal fibro-osseous tissue. Mainly, FD is found in children, and by adulthood it usually becomes quiescent. Our case showed FD of more than 14-year duration in the left maxilla. Our evaluation was that growth ceased in adulthood and had achieved the static stage. Because FD cases in elderly patients are rarely reported, we hereby present a monostotic FD case in a 65-year-old female. We presented sequential radiographic images and scintigraphic images of this case, and combined them with a literature review that emphasized the progression of the disease.

Pasteurized Tumoral Autograft for the Reconstruction of Monostotic Fibrous Dysplasia in Frontal Bone (저온 열처리 자가 종양골이식을 이용한 이마뼈의 단골성 섬유성이형성증의 재건)

  • Lee, Eui-Tai
    • Archives of Craniofacial Surgery
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    • v.11 no.2
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    • pp.91-94
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    • 2010
  • Purpose: For the best possible aesthetic reconstruction after craniofacial bone tumor resection, pasteurization has been adopted to devitalize neoplastic cells while maintaining osteoinductive properties and mechanical strength. This case report aims to demonstrate a long-term follow-up result of a monostotic fibrous dysplasia in frontal bone which was reconstructed by pasteurized tumoral autograft in situ. Methods: A 14-year-old girl presented with a hard, nontender, slowly growing mass of 6-year duration on her left supraorbital area. CT showed $5{\times}4{\times}3cm$ sized well defined bony mass confined to frontal bone with heterogeneous density. Tumor was excised completely through bicoronal approach and reimplanted to its original site after pasteurization at $60^{\circ}C$ for 30 minutes. The pathologic examination confirmed fibrous dysplasia. Results: She revisited our clinic 5 years later after suffering some assault on her face. On CT examination, pasteurized tumoral autograft was incorporated to host bone except the fractured upper orbital rim without any evidence of recurrence. She has been satisfied with the result. Conclusion: Pasteurization offers a simple, reliable, cosmetic, economic, and durable reconstruction method for craniofacial skeletal tumor. It has advantages of both biologic incorporation ability and mechanical strength without risk of recurrence. So, it should be considered as one of the primary options in benign as well as resectable malignant tumors of craniofacial skeleton.

Treatment of Fibrous Dysplasia (섬유성 골 이형성증의 치료)

  • Kim, Tai-Seuug;Yang, Mun-Seung;Cho, Jae-Lim
    • The Journal of the Korean bone and joint tumor society
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    • v.2 no.1
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    • pp.78-87
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    • 1996
  • The results of treatment of eighteen lesions of fibrous dysplasia which of them seventeen lesions were treated with surgery were reviewed at the Department of Orthopedic Surgery, College of Medicine, Hanyang University Hospital. We studied to evaluate the functional clinical results and the recurrence according to the type of disease, grafted bone, methods of treatment and location of lesion. We treated sixteen patients(five males and eleven females) and their mean age was 22.6 years. There was no association with skin pigmentation or dysfunction of endocrine system. Twelve patients had a monostotic pattern and four patients had a polyostotic pattern. Twelve lesions were treated with curettage and bone grafting and four lesions in the proximal femur were treated by internal fixation with curettage and bone grafting. One lesion was treated by en-bloc resection. There were eleven satisfactory results in twelve monostotic lesions and there were four satisfactory results in five polyostotic lesions, but the recurrence were four cases, respectively. The two unsatisfactory results were seen in two patients treated by autografting, but there were three recurrence of four lesions in autografting only, one of five in autografting and allografting, four of eight in allgrafting or xenografting. Four of six lesions in upper extremity were recurred after curettage and bone grafting and five of them showed satisfactory results. In pelvic and lower extremity lesions, the recurrence were occurred in two lesions after curettage and bone grafting and in two lesions after internal fixation and bone grafting. The recurrence does not always provide an unsatisfactory functional results and the recurrence showed higher rate in radiologic pattern of ground glass appearance with-out marginal sclerotic rim, but the recurrence according to grafted bone showed similar rates. Curettage and bone grafting is adequate for a symptomatic lesion and firm internal fixation is needed for a lesion in proximal femur. In use of grafted bone, xenograft or allograft may be preferable to autograft because of the disadvantage of autografting like a increased blood loss, prolonged operation times, etc.

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POLYOSTOTIC FIBROUS DYSPLASIA (다골성 섬유성 이형성증)

  • Lee Kang-Sook;Park Sang-Eok;Choi Karp-Shik
    • Journal of Korean Academy of Oral and Maxillofacial Radiology
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    • v.24 no.1
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    • pp.189-193
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    • 1994
  • Fibrous dysplasia is a benign fibro-osseous condition that is replacement of normal bone and marrow tissues by cellular fibrous tissue and immature bone, and it is divided into monostotic type and polyostotic type. Polyostotic fibrous dysplasia involves multiple bones, such as skull, jaw bones, femur and tibia. And it is also divided into two forms: the less severe Jaffe's type and the more severe Albright's syndrome. Clinically, it frequently occurs in the 2nd decade, and occurs more frequently in maxilla than in mandible. And the lesions of fibrous dysplasia tend to become static as skeletal maturity is reached. The authors experienced three cases of polyostosic fibrous dysplasia in the craniofacial area with the complaints of facial asymmetry due to painless swelling. And we discussed the clinical, radiological, and histopathological features of these cases with a brief review of the literatures.

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Maxillo-mandibular Contouring Surgery in Monostotic Fibrous Dysplasia Patients using Simulation Surgery

  • Kim, Dong-Young;You, Myoung-Sang;Ah, Kang-Min
    • Journal of International Society for Simulation Surgery
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    • v.3 no.2
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    • pp.60-63
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    • 2016
  • Fibrous dysplasia is quite a rare disease usually involving maxilla and mandible. Because of its benign clinical course, conservative contouring surgery has been recommended for facial deformity. 3D rapid prototype (RP) model gives a lot of informations before operation such as depth of drilling, area of resection and important anatomic structure. The purpose of this study was to report maxilla-mandibular contouring surgery in fibrous dysplasia patients. A total of 14 consecutive patients were included for surgical and esthetic evaluation. Among 14 patients, RP model study was performed in two patients with severe facial deformity. The other patients underwent contouring surgery under conventional methods. Surgical evaluation was performed with computed tomography scan before and after operation. Surgical resection was successful and patients were satisfied with the surgical results.

Fibrous Dysplasia of the Clivus

  • Kim, Ealmaan
    • Journal of Korean Neurosurgical Society
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    • v.48 no.5
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    • pp.441-444
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    • 2010
  • Fibrous dysplasia (FD) of craniofacial structures is well documented, however, its involvement of the clivus is seldom described. We report a case of clival FD in a young man who presented with headache localized to the occipital area. The radiological studies revealed a monostotic disease confined to the clivus, with typical findings of hypo intensity on magnetic resonance images and ground-glass density on computed tomography. The diagnosis of FD was confirmed on pathological examination of specimens taken through transsphenoidal surgery. The patient showed reduction of symptoms and no change of residual lesion on follow-up imaging taken 2.5 years later after surgery. This study includes clinical aspect, radiographic appearance, differential diagnosis and treatment strategy of this rare skull base lesion.

Endoscopic Decompression for Optic Neuropathy in McCune-Albright Syndrome

  • Noh, Jung-Hoon;Kong, Doo-Sik;Seol, Ho Jun;Shin, Hyung Jin
    • Journal of Korean Neurosurgical Society
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    • v.56 no.3
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    • pp.281-283
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    • 2014
  • McCune-Albright syndrome (MAS) is characterized by a triad of poly/monostotic fibrous dysplasia, caf$\acute{e}$-au-lait macules and hyperfunctioning endocrinopathies including human growth hormone excess. Acromegaly as a manifestation of endocrine hyperfunction with MAS is uncommon. Surgical excision may be challenging due to the associated severe fibrous dysplasia of the skull base. Through the endoscopic procedures, we treated a case of MAS presenting with compressive optic neuropathy due to fibrous dysplasia and acromegaly caused by growth hormone secreting pituitary adenoma. We reviewed the literature on GH excess in MAS to highlight its surgical and medical challenges.