통합 검색 | Korea Science

Dong Yeon Kim
- 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.
https://doi.org/10.7181/acfs.2023.00101 인용 PDF

Seungchul Baek;Byung Jun Kim
- 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.
https://doi.org/10.7181/acfs.2023.00255 인용 PDF

Al-Shaqsi, Sultan;Al-Bulushi, Taimoor;Al-Hinai, Qasim
- Archives of Plastic Surgery
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- 제45권6호
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- pp.578-582
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- 2018
Teratomas can occur in almost any region of the body and are the most common extragonadal germ cell childhood tumors. However, craniofacial teratomas are rare. Craniofacial teratomas can present unique features and cause significant functional and aesthetic concerns. There are complex lesions that can have components intra-cranially and extra-cranially. Therefore, their management requires significant multi-stage multidisciplinary surgical procedures. Herein, we present a case of craniofacial teratoma in a child with the phenotype of proboscis lateralis that highlights some of the pertinent point of the diagnosis and management of congenital neonatal teratomas.
https://doi.org/10.5999/aps.2017.01739 인용 PDF KSCI

정영수
- 대한구순구개열학회지
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- 제11권2호
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- pp.59-63
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- 2008
Craniofacial Centre at Children's Hospital Boston is a worldwide leader in the care of children and adolescents with craniofacial anomalies especially with cleft lip and/or cleft palate, which provides a team approach to the evaluation, diagnosis and treatment of children and adults with congenital (present at birth) or acquired facial deformities. This is staffed by an experienced team of clinicians, such as in oral and maxillofacial surgery, plastic surgery, neurosurgery, dentistry, audiology, speech and language pathology, genetics, psychiatry, otolaryngology, and social work, all with specialized training in the care of children with craniofacial anomalies. Here, there is a short introduction of history, attending surgeons, works, and sequential treatment for cleft lip/palate patients about this institution.
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