• Journal of the korean academy of Pediatric Dentistry
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• v.24 no.4
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• pp.751-757
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• 1997

Cherubism is an uncommon, benign fibrosseous lesion of the jaws that causes a progressive, painless, symmetrical expansion of the maxilla and mandible. It's autosomal dominance pattern of inheritance has been confirmed. A characteristic deformity is specific to this disease: hypertrophy of the mandible, swelling of cheeks and sometimes hypertrophy of maxilla with eyes tending to look up ; that looks like the Renaissance cherubs. It usually makes figures during childhood between 2-4 years of age and progresses until puberty, after which it spontaneously regresses 10 most cases. As a result of this case review, the management strategies for cherubismic children in standpoint of pediatic dentistry can be summarized as follows: 1. It can be detected early in childen through its characteristic clinical and radiographic features, which is confirmed by histopathological examination and familial history. 2. The supervision of arch space is required against its frequent sequelae, the early missing of primary teeth and eruption disorders of permanent teeth. 3. It is highly recommended to continue the periodic check-up with clinical and radiographic examination, leading to surgical intervention in cases of aggravation.

• Korean Journal of Head & Neck Oncology
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• v.37 no.1
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• pp.57-61
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• 2021

Fibrous dysplasia is a bone condition characterized by the replacement of normal bone tissue and the medullary cavity by abnormal fibrous tissues. Craniofacial fibrous dysplasia causes facial asymmetry compromising the aesthetics as well as vision and hearing. A 21-year-old male visited the clinic due to vertical orbital dystopia and exophthalmos that had developed over the previous 2 months. The patient was diagnosed with a fibrous dysplasia of the frontal, ethmoid bones and superior orbital wall. By a bicoronal incision on the scalp, the radical resection of the lesions was done. After harvesting the remaining frontal bone, we did the autogenous reconstruction using split calvarial bone graft. Postoperatively, the vertical orbital dystopia and exophthalmos significantly improved. The patient is satisfied with the surgical outcomes and has not reported any recurrence.

• Archives of Plastic Surgery
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• v.34 no.3
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• pp.403-405
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• 2007

Purpose: Malignant degeneration of fibrous dysplasia is an uncommon recognized complication of this disease. Especially, degeneration of fibrous dysplasia to malignant fibrous histiocytoma(MFH) in facial bone is rare and the publications had been limited. The purpose of this report is to share our experience. Methods: A 46-year-old patient with facial fibrous dysplasia visited our clinic for recent facial tingling and swelling. Malignant degeneration of fibrous dysplasia was suspected. Results: Total excision of the mass and adjacent facial bone was performed. Defect was immediately reconstructed with bone graft and bone cement. At a month follow up, metastasis was detected at ipsilateral parotid gland. Superficial parotidectomy and neck dissection was performed. The patient is currently taking chemotherapy. Conclusion: Because of the uncommon presentation of this entity, clinical course of treatment was dependent on other histological types of malignant degeneration. We report this case to share our experience.

• 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.

• 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 Academy of Oral and Maxillofacial Radiology
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• v.29 no.1
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• pp.55-63
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• 1999

The author observed and compared the radiographic features of 49 cases of the fibrous dysplasia and 14 cases of the ossifying fibroma in the osteoblastic or mature stage radiologically and histopathologically. The obtained results were as follows: 1. Fibrous dysplasia occurred most frequently in the 2nd decade, but ossifying fibroma in the 3rd and 4th decades, and both lesions occurred with slight predilection in females. 2. In most cases, chief complaints were painless facial swelling. And 61.1％ of fibrous dysplasia occurred in the maxilla, 92.9％ of ossifying fibroma in the mandible, and most of these lesions occurred in the premolar-molar region. 3. In the mandibular lesions, ossifying fibroma was shown more oval and round shape. but fibrous dysplasia was shown fusiform shape. 4. Fibrous dysplasia was shown homogeneously distributed. complete radiopaque shadow at 63％, and ossifying fibroma was shown concentric. mixed appearance of radiolucent and radiopaque shadow at 92.9％. 5. Fibrous dysplasia was entirely shown poorly outlined and blended to normal surrounding bone, but ossifying fibroma was shown well-defined border. 6. Cortical thinning and expansion were observed in these lesions. but degree of cortical expansion was more severe in ossifying fibroma than fibrous dysplasia. 7. Loss of lamina dura. tooth displacement. and displacement of mandibular canal were observed in both lesions. but root resorption was observed in ossifying fibroma only.

• The Journal of the Korean bone and joint tumor society
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• v.2 no.1
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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.

• The Journal of the Korean bone and joint tumor society
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• v.17 no.2
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• pp.73-78
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• 2011

Purpose: This study was aimed to evaluate the result of inlay cortical strut bone grafts for large cysts or cavitary bone lesions in long bones. Materials and Methods: Seven patients with large cyst or cavitary bony lesions were managed with curettage, allogeneic inlay cortical strut and cancellous bone grafts. Additional plate and screw fixations were performed in 6 patients. There were three SBCs, two FDs with secondary ABC changes, one FD and one post-cement spacer removal state. Three of them had pathologic fractures. Progression of bone healing and mechanical support and functional result were evaluated. The mean follow-up period was 25.4 months. Results: Incorporations into host bones were progressed in all, average 4.2 months in six metaphyseal regions and 5.8 months in five diaphyseal regions respectively. Full structural supports were achieved in all except one patient without any additional procedures. No allograft-related complication was developed. Mean functional score according to the MSTS criteria was 29.6 at last follow up. Conclusion: Inlay cortical strut graft provided additional mechanical stability and bone stock for screw purchase in large cyst or cavitary defects of long bones, which allow early mobilization and excellent functional outcome.

• Archives of Craniofacial Surgery
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• v.11 no.2
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• pp.91-94
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• 2010

Purpose: For the best possible aesthetic reconstruction after craniofacial bone tumor resection, pasteurization has been adopted to devitalize neoplastic cells while maintaining osteoinductive properties and mechanical strength. This case report aims to demonstrate a long-term follow-up result of a monostotic fibrous dysplasia in frontal bone which was reconstructed by pasteurized tumoral autograft in situ. Methods: A 14-year-old girl presented with a hard, nontender, slowly growing mass of 6-year duration on her left supraorbital area. CT showed $5{\times}4{\times}3cm$ sized well defined bony mass confined to frontal bone with heterogeneous density. Tumor was excised completely through bicoronal approach and reimplanted to its original site after pasteurization at $60^{\circ}C$ for 30 minutes. The pathologic examination confirmed fibrous dysplasia. Results: She revisited our clinic 5 years later after suffering some assault on her face. On CT examination, pasteurized tumoral autograft was incorporated to host bone except the fractured upper orbital rim without any evidence of recurrence. She has been satisfied with the result. Conclusion: Pasteurization offers a simple, reliable, cosmetic, economic, and durable reconstruction method for craniofacial skeletal tumor. It has advantages of both biologic incorporation ability and mechanical strength without risk of recurrence. So, it should be considered as one of the primary options in benign as well as resectable malignant tumors of craniofacial skeleton.