• 대한골관절종양학회지
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• 제1권2호
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• pp.249-253
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• 1995

The fibrous dysplasia is a progressive and disabling condition that lead to deformity, especially weight bearing bones. The morbidity that is associated with the polyostotic fibrous dysplasia is the recurrent fracture and deformity. Various methods of treatment had been failed to control this problem. We used osteotomy and reconstruction nailing for polyostotic fibrous dysplasia occured in the proximal part of right femur with varus deformity and reconstruction nailing in left femur without osteotomy, and interlocking intramedullary nailing in right tibia to prevent pathologic fracture. These methods brought a good result of bone union and full weight bearing ambulation after 1 year and 6 month follow up. We think these methods are useful methods to control refracture and deformity, so we reported this case with bibliographic reviews.

• 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.

• Imaging Science in Dentistry
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• 제30권2호
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• pp.149-154
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• 2000

Fibrous dysplasia is believed to be a hamartomatous developmental lesion of unknown origin. This disease is divided into monostotic and polyostotic fibrous dysplasia. Polyostotic type can be divided into craniofacial type, Lichtenstein-Jaffe type, and McCune-Albright syndrome. In this case, a 31-year-old female presented spontaneous loss of right mandibular teeth before 5 years and has shown continuous expansion of right mandibular alveolus. Through the radiographic view, the coarse pattern of the mixed radiopaque-lucent lesion was seen on the right mandibular body, and there was diffuse pattern of the mixed radiopaque-lucent lesion with ill-defined margin in the left mandibular body. In the right calvarium, the lesion had cotton-wool appearance. Partial excision for contouring, multiple extraction, and alveoloplasty were accomplished under general anesthesia for supportive treatment. Finally we could conclude this case was polyostotic fibrous dysplasia of cranio-maxillofacial area based on the clinical, radiologic finding, and histopathologic examination.

• 치과방사선
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• 제9권1호
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• pp.37-43
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• 1979

The authors observed 2 cases of fibrous dysplasia on the cranial and maxillofacial bones in 31, 28 aged sisters, who had come to the Infirmary of Dental College, Yonsei University. The serial roentgenograms and clinical findings had been taken and the results established as polyostotic fibrous dysplasia according to the findings in their images. The authors have obtained the results as follows: 1. Bony expansion of the mandible occured at 18 years of age and the facial assymetry appeared due to development of the lesions. 2. The traumatic history were not noted but weak tendency of familial history noted 3. Endocrine disturbances, hyperpigmentation on the skin and premature puberty in the infancy were not noted. 4. We have concluded these diseases as polyostotic fibrous dysplasia on the cranial and maxillofacial bones with weak familial tendency according to the findings

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

• Imaging Science in Dentistry
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• 제52권4호
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• pp.421-427
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• 2022

McCune-Albright syndrome (MAS) is a rare multisystem disorder characterized by a clinical triad of polyostotic fibrous dysplasia (FD), skin pigmentation, and hyperfunctioning endocrinopathies. A 42-year-old man visited our medical hospital for the treatment of intermittent headaches and was diagnosed with MAS with acromegaly. This patient showed various clinical features of MAS, including pituitary adenoma, polyostotic FD, and hypogonadotropic hypogonadism. The FD lesions showed characteristic radiographic features, such as widespread, sclerotic bony lesions in the cranial bones, mixed radiolucent-radiopaque multilocular lesions in the mandible, and radiolucent lesions in the axial and appendicular skeleton. Over the years, the patient had been hospitalized multiple times due to accidental bony fractures associated with the fragile bony state of FD. This report presents a retrospective description of a case of MAS, with a review of the relevant literature.

• Imaging Science in Dentistry
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• 제30권2호
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• pp.117-122
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• 2000

Fibrous dysplasia is a benign disorder of bone consisting of intramedullary proliferation of fibrous tissue and irregularly distributed, poorly developed bone. The disease manifests itself in the monostotic form in which only one bone is involved and the polyostotic form in which multiple bones at different sites are affected. We reported a extensive case of polyostotic fibrous dysplasia with involvement of craniofacial bones, mandible, ribs and extremities. A 18-year-old man showed remarkable right facial swelling who had been treated on right femur 3 years ago with a bone graft for pathologic fracture and he recognized facial swelling 5 years ago. Extraoral radiograms and computed tomogram showed diffuse sclerosis with a ground glass appearance of the most cranial bones, facial bones. The right mandibular lesion showed very expansile lesion with mottled appearance. Bone scans showed mutifocal increased uptakes in craniofacial bones, right mandible, bilaterally in ribs, humerus, femur, tibia and characteristic varus deformity of right femur (shepherd's crook defomity). This case showed exceptionally bilateral, extensive nature of bone lesion and didn't show any features of skin pigmentation and endocrine disturbances.

• Investigative Magnetic Resonance Imaging
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• 제11권1호
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• pp.49-53
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• 2007

섬유형성이상은 골모세포의 비정상적인 분화로 정상 골수와 해면골이 미숙골과 섬유성간질로 대치되는 유전이 되지 않는 골질환이다. 섬유형성이상은 다양한 영상소견을 보이며, 일반적으로 우연히 발견되며 정밀검사가 필요하지 않다. 그러나 섬유형성이상에서 병적골절의 합병증을 보일 수 있고 드물게 악성변이의 합병증을 가진다. 다골성섬유형성이상을 가진 44세 남자환자의 대퇴골에서 방사선소견상 골피질의 파괴와 자기공명영상에서 연부조직종괴를 가진 악성변이의 소견을 보였고, 수술로 절제된 조직학적 검사에서 연골아세포골육종으로 진단 받았다.

• Maxillofacial Plastic and Reconstructive Surgery
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• 제35권5호
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• pp.337-341
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• 2013

Fibrous dysplasia is a chronic developmental disease of the skeleton involving formation of immature bone. Involvement of facial bones can result in deformation of facial contour. Prominent cheek area is often treated with malarplasty, which has a variety of modifications, depending on the surgeon's preference. The authors report on a case of polyostotic fibrous dysplasia in which the patient's right cheek had a prominent appearance. The prominence was corrected with malarplasty without internal fixation. The soft nature of bone involved in fibrous dysplasia could provide greater flexibility for modification of the traditional surgery.

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