• 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.

• Maxillofacial Plastic and Reconstructive Surgery
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• 제42권
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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.

• 대한두경부종양학회지
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• 제37권1호
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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.

• 대한두개안면성형외과학회지
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• 제24권5호
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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.

• 대한두개안면성형외과학회지
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• 제11권2호
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• pp.91-94
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• 2010

저온 열처리법은 두개안면골 종양의 절제 후 재건에 있어 간단하고, 믿을만하며, 모양이 우수하고, 경제적이면서 또한 오랫동안 유지가 가능한 방법이다. 이 방법은 종양 재발의 걱정없이 생물학적으로 융합이 가능함과 동시에 기계적인 강도가 높은 장점을 가지고 있다. 따라서 두개안면골의 양성종양뿐만 아니라 절제가 가능한 악성종양의 경우에도 재건 시 우선적으로 고려해볼 만한 방법으로 사료된다.

• Brain Tumor Research and Treatment
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• 제6권2호
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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.

• 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.

• Journal of Korean Dental Science
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• 제8권1호
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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.

• 대한치과의사협회지
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• 제38권6호통권373호
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• pp.498-509
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• 2000

• Journal of International Society for Simulation Surgery
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• 제3권1호
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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.