• Archives of Craniofacial Surgery
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• v.24 no.2
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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.

• Imaging Science in Dentistry
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• v.30 no.2
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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.

• Imaging Science in Dentistry
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• v.30 no.2
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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.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.20 no.3
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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.

• Journal of Korean Dental Science
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• v.8 no.1
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• pp.36-40
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• 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
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• v.14 no.1_2
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• pp.124-134
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• 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.

• The Journal of the Korean bone and joint tumor society
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• v.15 no.1
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• pp.87-91
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• 2009

Fibrous dysplasia is a developmental benign disorder in which normal bone is replaced by fibrous tissue. Malignant transformation of fibrous dysplasia is a rare complication and more commonly occur in the polyostotic form than monostotic form. We report a case in which osteosarcoma developed in area of fibrous dysplasia of proximal femur after treating with curettage, wedge osteotomy, internal fixation, and bone graft. A review of the literature is presented.

• Archives of Craniofacial Surgery
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• v.24 no.5
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• pp.244-249
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• 2023

Fibrous dysplasia (FD) is a rare skeletal disorder characterized by abnormal fibro-osseous connective tissue replacing normal bone. Despite its benign behavior, craniofacial FD can cause morphological disfigurement, headache, and even blindness as a result of the produced mass effect. Surgical resection is recommended when the patient shows apparent clinical symptoms or aggravating facial asymmetry. Postoperative complications have been reported, such as hematoma, surgical site infection, abscess formation, resorption of the bone graft used for reconstruction, and recurrence. An aneurysmal bone cyst (ABC) is a rare benign bony lesion that can occur secondary to preexisting bone tumor. Secondary ABCs in craniofacial FD are extremely rare in the literature, accounting for less than 30, all of which are either case reports or series. We report an extremely rare case of symptomatic secondary ABC arising from craniofacial FD that had been misdiagnosed with abscess formation or recurrence and was surgically removed. Notably, 17 years elapsed between the primary surgery and the complication of secondary ABC. The patient underwent total removal of secondary ABC. After surgery, symptoms were relieved, with no recurrence observed during a 6-month follow-up.

• Journal of Korean Academy of Oral and Maxillofacial Radiology
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• v.21 no.2
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• pp.261-273
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• 1991

This study was undertaken to document and better defined this condition to help clarify this clinical and radiographical appearances by the analysis of clinical and radiographical features of fibro-osseous lesions in the jaws. A study was made of a series of 128 cases with fibro-osseous lesions. The obtained results were as follows. 1. Fibrous dysplasia of the jaws occurred with equal predilection for males and females. But the females occurred in 68% of cemento-ossifying fibroma and 75% of periapical cemental dysplasia. 2. 43% of fibrous dysplasia and 32% of cemento-ossifying fibroma occurred in the 2nd decades and 33% of periapical cemental dysplasia in 5th decades. 3. 62% of fibrous dysplasia occurred in the maxilla, 73% of cemento-ossifying fibroma in mandible, 90% of periapical cemental dysplasia in mandible. 4. 98% of fibrous dysplasia occurred in premolar-molar region, 77% of cemento-ossifying fibroma in molar region, 68% of periapical cemental dysplasia in incisor region. 5. In serial radiographic features, mature stage were 55% of fibrous dysplasia, 45% of cemento-ossifying fibroma, 59% of periapical cemental dysplasia. 6. 87% of fibrous dysplasia had monostotic lesion, 67% of periapical cemental dysplasia had multiple lesions. 7. In fibrous dysplasia and cemento-ossifying fibroma, migration of tooth occurred in 61.7% and 36.4%, retention of tooth occurred in 4.3% and 9.1%, loss of lamina dura occurred in 6.4% and 9.1%, and root resorption had not occurred in fibrous dysplasia, but occurred in 18% of cemento-ossifying fibroma, displacement of mandibular canal occurred in 14.9% and 31.8%.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.12 no.3
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• pp.68-73
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• 1990

Fibrous dysplasia is an idiopathic skeletal disorder in which medullary bone is replaced and disturbed by poorly organized, structually unsound fibroosseous tissue, which may produce cortical expansion. When facial bones are involed, 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 arises as a resujlt of trauma. It occurs predominantly in infant, adolescent females and runs a variable clinical course. When several bones are involed, it tends to be unilateral. Involements of alveolar bone may produce displacement of teeth with malocclusion, or loss of teeth, or both. Radiographycally, it shows an indistinctly delimited osteolytic defect with a bubble - like pattern, but without a sclerotic rim. The preferred treatment is almost always surgery. If the lesion is extensive, surgical intervention with use of recontouring procedures aimed at the correction of esthetic or funtional disturbances is preferred treatment. Now, we present a case of fibrous dysplasia on the left maxilla and the zygoma treated by bony contourign via hemicoronal flap and intraoral approach with good results.