• Investigative Magnetic Resonance Imaging
• /
• 제13권2호
• /
• pp.203-206
• /
• 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
• /
• 제11권2호
• /
• pp.188-193
• /
• 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.

• 대한두개안면성형외과학회지
• /
• 제24권2호
• /
• pp.41-51
• /
• 2023

Fibrous dysplasia is an uncommon genetic disorder in which bone is replaced by immature bone and fibrous tissue, manifesting as slow-growing lesions. Sporadic post-zygotic activating mutations in GNAS gene result in dysregulated GαS-protein signaling and elevation of cyclic adenosine monophosphate in affected tissues. This condition has a broad clinical spectrum, ranging from insignificant solitary lesions to severe disease. The craniofacial area is the most common site of fibrous dysplasia, and nine out of 10 patients with fibrous dysplasia affecting the craniofacial bones present before the age of 5. Surgery is the mainstay of treatment, but the technique varies according to the location and severity of the lesion and associated symptoms. The timing and indications of surgery should be carefully chosen with multidisciplinary consultations and a patient-specific approach.

• Maxillofacial Plastic and Reconstructive Surgery
• /
• 제14권1_2호
• /
• pp.124-134
• /
• 1992

Fibrous dysplasia is a benign pathologic condition of bone in which fibrous tissue gradually expands and replaces normal bone into fibro-osseous lesion. It is a primary developmental abnormality of bone-forming mesenchyme in origin. This study shows clinical history, radiological and histopathological feature of fibrous dysplasia with the intention of establishing correct diagnosis, treatment plan and evaluation of prognosis. This paper reviews and summarizes the materials from 57 fibrous dysplasias submitted to the Department of Oral and Maxillofacial Surgery in College of Dentistry, Seoul National University. Conclusions obtained were as following : 1. Fibrous dysplasia developed mainly in teenagers and shows female predeliction. 2. Fibrous dysplasia developed much on the maxilla 3. Monostotic fibrous dysplasia was most popular form. 4. Main symptom of fibrous dysplasia was painless swelling. 5. Radiological feature of fibrous dysplasia was ground-glass appearance, 6. Histopathological feature of fibrous dysplasia was irregular immature bony trabeculae(woven bone). 7. Treatment of fibrous dysplasia was mainly conservative contouring surgery.

• 치과방사선
• /
• 제24권1호
• /
• pp.189-193
• /
• 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 Korean Dental Science
• /
• 제8권1호
• /
• pp.36-40
• /
• 2015

Fibrous dysplasia is a bone disorder characterized by progressive replacement of normal bone by fibrous bone tissue. Common involving sites of fibrous dysplasia are the skeletal system including long bones, ribs, craniofacial bones and the pelvis. If maxilla were affected by fibrous dysplasia, antrum is almost always involved. And fibrous dyplasia in maxillary sinus were followed the shape of bone. In our case, the lesion involves antrum but, its shape was different from typical fibrous dysplasia pattern of maxillary sinus. Therefore we report a case of monostotic pediculated fibrous dysplasia in the maxillary sinus with a review of literature.

• Maxillofacial Plastic and Reconstructive Surgery
• /
• 제20권3호
• /
• pp.232-236
• /
• 1998

Fibrous dysplasia is a benign pathologic condition of bone which medullary bone is replaced and disturbed by poorly organized, structually unsound fibro-osseous tissue. When facial bones are involved, considerable esthetic deformity may result. The term monostotic fibrous dysplasia has been applied when one bone is involved : when more than one bone is affected, the term polyostotic used. The polyostotic form may be accomplished by pigmented skin lesion (Jaffe type), or by pigmented skin lesions with endocrine disturbance (Albright syndrome). No general agreement exists on the cause of fibrous dysplasia. A few authors have suggested that fibrous dysplasia as a result of trauma. It occurs predominantly in infant, adolescent females and runs a variable clinical coures. When several bones are involved, it tends to be unilateral. Involvements of alveolar bone may produce displacement of teeth with malocclusion, or loss of teeth, or both. Now, we will present a case of fibrous dysplasia on the left facial region treated by conservative contouring surgery.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
• /
• 제35권5호
• /
• pp.304-309
• /
• 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.

• Journal of International Society for Simulation Surgery
• /
• 제3권2호
• /
• pp.60-63
• /
• 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
• /
• 제56권3호
• /
• pp.281-283
• /
• 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.