• 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.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.40
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• pp.38.1-38.9
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• 2018

Background: Fibrous dysplasia (FD) is a benign bone lesion characterized by the progressive replacement of normal bone with fibro-osseous connective tissue. The maxilla is the most commonly affected area of facial bone, resulting in facial asymmetry and functional disorders. Surgery is an effective management option and involves removing the diseased bone via an intraoral approach: conservative bone shaving or radical excision and reconstruction. Case presentation: This case report describes a monostotic fibrous dysplasia in which the patient's right midface had a prominent appearance. The asymmetric maxillary area was surgically recontoured via the midfacial degloving approach under general anesthesia. Follow-up photography and radiographic imaging after surgery showed the structures were in a stable state without recurrence of the FD lesion. Furthermore, there were no visible scars or functional disability, and the patient reported no postoperative discomfort. Conclusions: In conclusion, the midfacial degloving approach for treatment of maxillary fibrous dysplasia is a reliable and successful treatment option. Without visible scars and virtually free of postoperative functional disability, this approach offers good exposure of the middle third of the face for treatment of maxillary fibrous dysplasia with excellent cosmetic outcomes.

• Journal of Korean Foot and Ankle Society
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• v.15 no.1
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• pp.32-35
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• 2011

Fibrous dysplasia is a benign disease that causes replacement of the medullary bone with fibrous tissue in one or more bones. Long bone like femur, tibia and fibular are often affected and occurring under 30 years old. We report a case of two solitary lesions of fibrous dysplasia with pathologic fracture treated with bone curettage, bone graft, plate fixation, who complains of lower leg pain.

• Archives of Plastic Surgery
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• v.33 no.2
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• pp.259-262
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• 2006

Fibrous dysplasia(FD) of the bone is a slowly progressive, benign disease of unknown cause where normal architectures are replaced with fibrous and osteoid tissue. FD of the maxilla usually manifests as a bony enlargement with painless swelling and bone deformity, contouring to facial asymmetry. The lesion may involve the nasal fossae, orbits, or alveolus bone, causing diverse functional disturbance. Treatment options range from shaving to total maxillectomy and reconstruction depending on the presenting symptoms. Shaving, partial maxillectomy and maxillary sinus formation was performed in 5 patients with fibrous dysplasia in the past 2 years. Follow up period ranged from 1 month to 11 months. Aesthetic appearance, CT findings, and relief from symptoms were compared. In all patients, facial asymmetry was restored to symmetry and nasal obstructive symptoms were improved. With this procedure, expansion of the lesion will be controlled until puberty, preventing the development of new functional disturbances. After puberty, no further treatment can be anticipated due to the growth arrest inherent to the disease.

• Journal of Korean Dental Science
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• v.6 no.1
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• pp.34-40
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• 2013

Fibrous dysplasia is a benign fi bro-osseous lesion wherein normal bone is replaced with an excessive proliferation of cellular fibrous connective tissue intermixed with irregular bony trabeculae. Fibro-osseous lesions like fibrous dysplasia are often associated with non-epithelial cysts, such as simple bone cyst. The etiologic and pathogenic relationships between fi brous dysplasia and simple bone cyst have not been conclusively established. Nonetheless, the mechanism of cyst formation in fibro-osseous lesion associated with simple bone cyst can be said to differ from that of the typical simple bone cyst of the jaws. This article reports a case of bilateral lesions including fi brous dysplasia and simple bone cyst on each site and reviews the pathogenesis of cyst formation in the fibro-osseous lesion.

• The Journal of the Korean dental association
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• v.17 no.1 s.116
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• pp.19-23
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• 1979

An instance of facial asymmetry due to fibrous dysplasia occurred on left maxilla in 18-year-old girl was observed. After operation by partial resection and curettage, she gained good facial profile and had no complication.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.23 no.4
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• pp.366-375
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• 2001

Bone shaving for surgical correction is general method in facial asymmetrical patient with fibrous dysplasia. Therefore, decision of bone shaving amount on the preoperative planning is very difficult for improvement of ideal occlusal relationship and harmonious face. Preoperative planning of facial asymmetry with fibrous dysplasia is generally confirmed by the simulation surgery based on evaluation of clinical examination, radiographic analysis and analysis of facial study model. However, the accurate postoperative results can not be predicted by this method. By using the computed tomography based RP(rapid prototyping) model, simulation of facial skeleton can be duplicated and 3-dimensional simmulation surgery can be perfomed. After fabrication of postoperative study model by preoperactive bone shaving, preoperative and postoperactive surgical index was made by omnivaccum and clear acrylic resin. Amount of bone shaving is confirmed by superimposition of surgical index at the operation. We performed the surgical correction of facial asymmetry patients with fibrous dysplasia using surgical index and prototyping model and obtained the favorable results.

• Journal of the Korean Society of Radiology
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• v.82 no.5
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• pp.1346-1351
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• 2021

Mazabraud syndrome is a rare benign disease that is accompanied by polyostotic fibrous dysplasia and intramuscular myxoma. Malignant transformation of fibrous dysplasia occurs in approximately 1% of cases. To date, only eight cases of malignant transformation, of fibrous dysplasia to osteosarcoma, in Mazabraud syndrome have been reported worldwide. The authors report the first case of osteosarcomatous transformation in a patient with Mazabraud syndrome in the Republic of Korea, focusing on imaging findings.

• The Journal of the Korean bone and joint tumor society
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• v.14 no.1
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• pp.62-67
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• 2008

Malignant degeneration of fibrous dysplasia is rare and involves transformation into osteosarcoma, fibrosarcoma and chondrosarcoma. The most frequent sites involved in malignant transformation were craniofacial bones, proximal femur, humerus, pelvis, tibia and scapula in a decreasing order of frequency. An 41-year-old man with a history of polyostotic fibrous dysplasia presented with increasing left arm pain. Plain radiograph showed expansile destructive lesion along the humeral shaft. As initial biopsy report was low grade chondrosarcoma, he underwent marginal resection. However, he developed local recurrence 7 month later and subsequent pathologic finding was chondroblastic osteosarcoma. We report one case of secondary chondroblastic osteosarcoma from polyostotic fibrous dysplasia initially misdiagnosed as low grade chondrosarcoma that caused fallacy in treatment strategy.

• 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.