• Investigative Magnetic Resonance Imaging
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• 제13권2호
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• pp.203-206
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• 2009

섬유성 이형성증은 원인불명의 섬유조직의 양성질환으로 정상적인 해면골이 섬유조직과 불규칙적인 골소주의 증식에 의해 점진적으로 대체되는 특징을 가지며 단골성 또는 다골성으로 발생한다. 단골성의 경우 주로 늑골, 대퇴골, 경골 등에서 흔히 발생하지만 손에 발생하는 경우는 매우 드문 것으로 알려져 있다. 저자들은 13세 남아의 중수골에 발생한 단골성 섬유성 이형성증의 단순X선촬영과 자기공명영상의 소견을 중심으로 증례보고 하고자 한다.

• 대한골관절종양학회지
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• 제11권2호
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• pp.188-193
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• 2005

섬유성 이형성증은 주로 장관골을 침범하는 것으로 알려졌으며, 수질골이 섬유성 조직으로 대치되는 질환을 가리킨다. 단골성 섬유성 이형성증이 다골성에 비해 흔한 것으로 알려져 왔다. 문헌 고찰에 따르면, 단골성 섬유성 이형성증이 척추를 침범한 례는 저자의 보고 이전에 23례가 보고되어 있다. 저자는 요통을 주소로 내원하였던 34세 남자환자에서 발생한 제3요추를 침범한 단골성 섬유성 이형성증의 예를 경험하였기에 이에 대한 방사선학적 소견 및 치료 경과에 관한 보고를 문헌고찰과 함께 보고하고자 본 논문을 계획하였다.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• 제35권5호
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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.

• Imaging Science in Dentistry
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• 제46권4호
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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.

• 대한두개안면성형외과학회지
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• 제11권2호
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• pp.91-94
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• 2010

저온 열처리법은 두개안면골 종양의 절제 후 재건에 있어 간단하고, 믿을만하며, 모양이 우수하고, 경제적이면서 또한 오랫동안 유지가 가능한 방법이다. 이 방법은 종양 재발의 걱정없이 생물학적으로 융합이 가능함과 동시에 기계적인 강도가 높은 장점을 가지고 있다. 따라서 두개안면골의 양성종양뿐만 아니라 절제가 가능한 악성종양의 경우에도 재건 시 우선적으로 고려해볼 만한 방법으로 사료된다.

• 대한골관절종양학회지
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• 제2권1호
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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.

• 치과방사선
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• 제24권1호
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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.

• Journal of International Society for Simulation Surgery
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• 제3권2호
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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.

• Journal of Korean Neurosurgical Society
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• 제48권5호
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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.

• Journal of Korean Neurosurgical Society
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• 제56권3호
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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.