• 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.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.42
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• pp.31.1-31.7
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• 2020

Background: Fibrous dysplasia (FD) is a rare, sporadic, and benign congenital condition in which normal cancellous bone is replaced by fibro-osseous tissue with immature osteogenesis. FD localized in the cranial and facial bones is called craniofacial fibrous dysplasia (CFD). Cystic degeneration in CFD cases is rare; cystic degeneration appearing in both the maxilla and the mandible FD lesion is even rarer. The aim of this article was to report a case of fibrous dysplasia of the mandible and maxilla complicated by nonspecific cystic degeneration. Case presentation: A 30-year-old woman presented with a rare case of non-specific cystic degeneration in a mandible and maxilla FD lesion that occurred 11 years after surgery. She was diagnosed with polyostotic CFD and underwent maxillary and mandibular bone contouring. Cyst enucleation under general anesthesia was performed in the mandibular region due to pain and discomfort. Conclusions: In cases involving non-aggressive and non-invasive FD cystic degeneration in focal areas, conservative treatment is recommended. However, if cystic degeneration of FD develops rapidly and causes discomfort, pain, or dysfunction, surgical treatment should be considered.

• 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 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.

• 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.

• Brain Tumor Research and Treatment
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• v.6 no.2
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• pp.86-91
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• 2018

Aneurysmal bone cyst (ABC) is a rare non-neoplastic bone lesion that involves mostly the long bones and vertebrae and may occur very rarely in the craniofacial bones. ABCs may occur as secondary bony pathologies in association with various benign and malignant bone tumors and with fibrous dysplasia (FD). FD is a common non-neoplastic bony pathology mostly affecting craniofacial bones. Secondary ABC occurring in craniofacial FD is extremely rare, with only approximately 20 cases reported in the literature to date. Here, we report on a case of secondary ABC in a 25-year-old woman who has had a craniofacial deformity for over 10 years and who presented to us with a rapidly growing painful pulsatile mass in the right frontal region that began over 2 months prior to admission. On thorough examination of computed tomography and magnetic resonance imaging brain scans taken at two-month interval, an aggressive, rapidly enlarging ABC, arising from the right frontal FD, was diagnosed. The patient underwent preoperative embolization followed by gross total resection of the ABC and cranioplasty. The 6-month follow up showed no recurrence of the ABC, nor was any progression of the FD noticed.

• Journal of Korean Academy of Oral and Maxillofacial Radiology
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• v.24 no.1
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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.

• 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.

• The Journal of the Korean dental association
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• v.38 no.6 s.373
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• pp.498-509
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• 2000

• Journal of International Society for Simulation Surgery
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• v.3 no.1
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• pp.33-35
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• 2016

Fibrous dysplasia(FD) is a disorder in which normal bone is replaced with pathologic tissue. When occurring in craniofacial regions, the zygomaticomaxillary complex is most commonly affected and this pathologic lesion results in facial asymmetry. and By using computer-assisted virtual simulation, precise maxillofacial contouring was achieved for harmonious facial morphology and the surgical procedure was simplified and the surgery brought satisfactory results in terms of both esthetics and functionality.