• 제목/요약/키워드: Fibrous Dysplasia

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Central giant-cell granuloma in a patient with neurofibromatosis type 1: 7 years of follow-up

  • Michelle Briner Garrido;Rohan Jagtap;Christopher D. Matesi;Vivian Diaz;John Hardeman;Anita Gohel
    • Journal of the Korean Association of Oral and Maxillofacial Surgeons
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    • v.50 no.1
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    • pp.49-55
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    • 2024
  • Neurofibromatosis type 1 (NF1) is an autosomally dominant tumor suppressor syndrome and multisystem disease. Central giant-cell granulomas (CGCGs) can be seen in patients with NF1. A 21-year-old female was diagnosed with two CGCGs, one in the mandible and then one in the maxilla, in a 7-year period. Increased incidence of CGCGs in NF1 patients was thought to be caused by an underlying susceptibility to developing CGCG-like lesions in qualitatively abnormal bone, such as fibrous dysplasia. However, germline and somatic truncating second-hit mutations in the NF1 gene have been detected in NF1 patients with CGCGs, validating that they are NF1-associated lesions. Oral manifestations in patients with NF1 are very common. Knowledge of these manifestations and the genetic link between NF1 and CGCGs will enhance early detection and enable optimal patient care.

Garre's osteomyelitis of the mandible managed by nonsurgical re-endodontic treatment

  • Heegyun Kim;Jiyoung Kwon;Hyun-Jung Kim;Soram Oh;Duck-Su Kim;Ji-Hyun Jang
    • Restorative Dentistry and Endodontics
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    • v.49 no.2
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    • pp.13.1-13.7
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    • 2024
  • Chronic osteomyelitis with proliferative periostitis, known as Garre's osteomyelitis, is a type of osteomyelitis characterized by a distinctive gross thickening of the periosteum of bones. Peripheral reactive bone formation can be caused by mild irritation or infection. Garre's osteomyelitis is usually diagnosed in children and young adults, and the mandible is more affected than the maxilla. The following is a case report of a 12-year-old female patient with Garre's osteomyelitis of the mandible due to an infection of a root canal-treated tooth. Without surgical intervention, the patient's symptoms were relieved through nonsurgical root canal re-treatment with long-term calcium hydroxide placement. A cone-beam computed tomography image obtained 6 months after treatment completion displayed complete healing of the periapical lesion and resolution of the peripheral reactive buccal bone. Due to the clinical features of Garre's osteomyelitis, which is characterized by thickening of the periosteum, it can be mistaken for other diseases such as fibrous dysplasia. It is important to correctly diagnose Garre's osteomyelitis based on its distinctive clinical features to avoid unnecessary surgical intervention, and it can lead to minimally invasive treatment options.

Use of the Autogenous Calvarial Bone in Craniofacial Bone Graft (두안면부의 골이식시 자가 두개골의 이용)

  • Woo, Sang-Hyun;Jeong, Jae-Ho;Lee, Dae-Hoon;Choi, See-Ho;Seul, Jung-Hyun
    • Journal of Yeungnam Medical Science
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    • v.4 no.2
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    • pp.75-81
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    • 1987
  • Bone grafts are an integral and important aspect of craniofacial reconstruction. Ribs, tibia, and iliac bone have traditionally used as donor site but each of these has various problems, however using to the calvarial bone as a donor site has several advantage. These are as follows ; there are abundance of material, easy to reach the donor site through coronal incision, minimal pain of donor area, less functional inability, shorter hospitalization, no need of immobilization, hidden scar at donor site, no secondary deformity and appropriate curvature obtained properly selected. From march to December 1987, we experienced three cases of autogenous calvarial bone graft such as congenital saddle nose deformity, fibrous dysplasia on the right side frontal bone, and deviated nose. The results were very excellent without any significant complication. The detail technique of autogenous calvarial bone graft and its advantages compared with the traditional methods of bone grafts are discussed.

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Modified Orbitozygomatic Approach without Orbital Roof Removal for Middle Fossa Lesions

  • Lopez-Elizalde, Ramiro;Robledo-Moreno, Edgar;O'Shea-Cuevas, Gabriel;Matute-Villasenor, Esmeralda;Campero, Alvaro;Godinez-Rubi, Marisol
    • Journal of Korean Neurosurgical Society
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    • v.61 no.3
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    • pp.407-414
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    • 2018
  • Objective : The purpose of the present study was to describe an OrBitoZygomatic (OBZ) surgical variant that implies the drilling of the orbital roof and lateral wall of the orbit without orbitotomy. Methods : Design : cross-sectional study. Between January 2010 and December 2014, 18 patients with middle fossa lesions underwent the previously mentioned OBZ surgical variant. Gender, age, histopathological diagnosis, complications, and percentage of resection were registered. The detailed surgical technique is described. Results : Of the 18 cases listed in the study, nine were males and nine females. Seventeen cases (94.5%) were diagnosed as primary tumoral lesions, one case (5.5%) presented with metastasis of a carcinoma, and an additional one had a fibrous dysplasia. Age ranged between 27 and 73 years. Early complications were developed in four cases, but all of these were completely resolved. None developed enophthalmos. Conclusion : The present study illustrates a novel surgical OBZ approach that allows for the performance of a simpler and faster procedure with fewer complications, and without increasing surgical time or cerebral manipulation, for reaching lesions of the middle fossa. Thorough knowledge of the anatomy and surgical technique is essential for successful completion of the procedure.

Juxtacortical Osteogenic Sarcoma of Chondroblastic Type on the Maxilla (상악골에 발생된 연골형성현 측피질골육종)

  • Choi Sun-Young;Choi Eun-Suk;Koh Kwang-Joon
    • Journal of Korean Academy of Oral and Maxillofacial Radiology
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    • v.28 no.2
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    • pp.539-563
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    • 1998
  • 39-year-old female had been treated for the exophytic mass on buccal aspect of the left, maxillary posterior area 2 years and 8 months ago. Tentative diagnosis was obtained as fibrous dysplasia on clinical and radiographic examinations and histopathologic findings revealed as osteochondroma after bone trimming at that time. She revisited for the treatment of recurred lesions. We reviewed this case with clinical. radiologic and histopathologic standpoints retrospectively, and came to a conclusion that the tumor primarily occurred was juxtacortical osteogenic sarcoma and recurred due to inadequate treatment and then expanded over intramedullary. This case shows that the diagnosis of osteosarcoma should take account of the patient history, clinical. radiographic and histopathologic findings and it requires attentive follow up check. Retrospectively reviewed results were as follows ; At first visit, oral examination revealed a bony hard swelling on the buccal aspect of the left maxillary posterior area. Radiographically, a dense radiopaque mass was noted on the site. The lesion showed hot uptake of /sup 99m/Tc-MDP. Histopathologic diagnosis was done as osteochondroma, but it was considered as osteogenic sarcoma when compared with the recurrent lesion. When she revisited for the treatment of multiple bony swelling on the left maxilla, radiograms showed typical features of malignancy such as widening of periodontal ligament space and sunray appearace, and coincided with benign characters as follows; relatively well circumscribed lesion and expansion and displacement of the adjacent structures. Finally, histopathologic findings of the lesion was well differentiated chondroblastic osteogenic sarcoma.

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Surgical Approach of the Clivus and Anterior Cervical Spine (사대 및 전경추부의 수술적 접근법)

  • Tae Kyung;Lee Hyung-Seok;Park Chul-Won;Kim Kyung-Rae;Bak Koang-Heum;Kim Joo-Mook
    • Korean Journal of Head & Neck Oncology
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    • v.15 no.2
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    • pp.172-176
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    • 1999
  • Background: An increasing number of traumatic, infectious, degenerative, benign, and malignant lesions of the clivus and cervical spine are treated by head and neck surgeons. Surgical techniques vary according to the exact level of the lesion. Objectives: The purpose of this article are to introduce the surgical approach and to discript our experience and to analyze the advantage of the each methods. Materials and Methods: Transpalatal approach in clivus chordoma, transoral approach in C2 fibrous dysplasia, trans mandibular approach in clivus meningioma and transcervical approach in neulilemmoma were reviewed from the medical record. Results: Postoperative complications occured. Otitis media with effusion were observed in transoral and trans mandibular approach. Cranial nerve paralysis was observed in transcervical approach. But other complications such as swallowing difficulty persisting over 6 weeks, malunion of mandible, orocutaneous fistula, hemorrhage from major vessel were not observed. Conclusion: The various surgical techniques to be described herein serve to give the best exposure of each level of the lesions. Therefore head and neck surgeon plays a major role in the treatment of lesion in the clivus and anterior cervical spine according to the lower incidence of postoperative morbidity.

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A CLINICO-RADIOLOGIC STUDY OF THE VARIOUS LESIONS IN THE ORAL AND MAXILLOFACIAL REGION OF CHILDREN (소아의 구강악안면 영역에서 발생된 각종 병변의 임상방사선학적 연구)

  • Choi Soon-Chul;Lee Young-Ho;Park Tae-Won;You Dong-Soo
    • Journal of Korean Academy of Oral and Maxillofacial Radiology
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    • v.25 no.2
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    • pp.513-520
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    • 1995
  • Two thousand-two hundred-forty-three cases of various lesions of the oral and maxillofacial region in children under 16 years of age were reviewed. The lesions were classified by 10 groups; osteomyelitis, cysts, benign tumors, malignant tumors, fibro-osseous lesions, developmental disturbances, antral lesions, TMJ lesions, salivary gland lesions, and other lesions. The obtained results were as follows; 1. Developmental disturbances, especially cleft alveolus and palate, was the most common lesions (25.7%), followed by cysts (22.5%), antral lesions (12.7%), benign tumors (11.9%) and osteomyelitis (9.3%). 2. With the time, the incidence of osteomyelitis and malignant tumors has been decreased, but that of developmental disturbances, cysts and antral lesions has been increased. 3. The sex distribution was relatively equal for the entire series, with male predominence in cysts, malignant tumors, developmental disturbanes and salivary gland lesions and with female predominence in TMJ lesions. 4. Children with malignant tumors and osteomyelitis were somewhat younger and those with TMJ lesions were somewhat older. 5. Heading the list of each group except osteomyelitis was dentigerous cyst, odontoma, malignant lymphoma, fibrous dysplasia, cleft alveolus and palate, inflammatory change, degenerative change of condylar process, sialolithiasis and simple bone cyst respectively.

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Scintigraphic Findings of Fibrous Dysplasia (섬유성 골 이형성증의 골 신티그라피 소견)

  • Kim, Jong-Ho;Kim, Jong-Soon;Han, Seung-Soo;Kim, Sang-Eun;Choi, Chang-Woon;Lee, Dong-Soo;Chung, June-Key;Lee, Myung-Chul;Koh, Chang-Soon;Kang, Heung-Sik
    • The Korean Journal of Nuclear Medicine
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    • v.25 no.2
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    • pp.219-226
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    • 1991
  • 골 신티 그라피는 섬유성 골 이형성증의 초기 병소와 다골성 형 섬유성 골 이형성증의 진단에 예민한 검사 방법이나, 양성 또는 악성 골 병변과의 감별진단에 어려움이 있었다. 이에 저자들은 조직학적으로 확진된 섬유성 골 이형성증 환자 17명에서 (단골성 형 12명, 다골성 형 : 5명) $^{99m}Tc-MDP$ (20 mCi 또는 7,400 MBq) 주사후 $2\sim4$시간에 전면, 후면 전신상과 부분상을 얻은 30부위 병소를 X선 사진과 비교분석 하였으며 CT, MRI 영상과 병리조직 소견을 얻었다. 병소 침습 부위별로는 대퇴골-10예, 경골-4, 요골-2, 늑골-2, 척추골-2, 후두골-2, 장골-2, 두정골-1, 하악골-1, 접형골-1, 견갑골-1, 쇄골-1, 척골-1예 였으며 5명의 다골성 형에서는 :우 대퇴골과 우 비골 : 양측 말단 대퇴골 :좌 경골과 좌 대퇴골 :우 요골과 우 장골 양측 대퇴골, 양측 경골, 접형골, 두정골, 척추골, 늑골과 장골이었다. 골 신티 그라피 소견상 30예중 28예에서(28/30, 93.3%) 방사능 섭취 증가를 보였으며 2예에서(2/30, 6.7%)는 정상 방사능 섭취 소견을 보였으나 각각은 X선상 불투명 유리상과 골흡수 병변을 보였다. 하악골의 골 3상 스캔상 병소에 현저한 혈류 증가가 관찰되었다. X선 소견상 30예중 11예에서 (l1/30, 36.7%) 불투명 유리상의 병변을, 골 변형을 동반한 1예를 포함한 18예에서는 (18/30, 60.0%) 골흡수 병변을 보였으며, 1예의 늑골 병변은 정상소견을 보였다(1/30, 3.3%). 이상에서 골 신티 그라피 만으로 섬유성 골 이형성증을 진단하는데는 주의를 요하나 골 대사의 동적 측면인 혈류와 골 재형성 양상 특히, 초기 병변과 단골성 형 침습에서 다골성 형을 진단하는데 필수적이며 결론적으로 골 신티 그라피와 X선 촬영등은 섬유성 골 이형성증 진단에 상호보완적인 검사 방법으로 사료된다.

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Treatment of Benign Bone Tumor with Xenograft (이종골 이식을 이용한 양성 골 종양 치료)

  • Kim, Han-Soo
    • The Journal of the Korean bone and joint tumor society
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    • v.1 no.2
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    • pp.189-193
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    • 1995
  • The authors reviewed 49 cases(48 patients) of benign bone tumor who had surgical treatment with xenograft at department of orthopedic surgery, Seoul National University Hospital from May, 1980 to May, 1994. Materials consist of 21 males and 27 females. The mean age at operation was 20.1 years(range : 4 -55 years) and the mean follow up period was 25.4 months(range : 7 - 85 months). We did xenograft only in 34 cases and xenograft mixed with autograft in 15 cases(14 cases, from ilium, 1 case from femur). The used materials for xenograft were $Lubboc^{(R)}$ in 29 cases, $Surgibone^{(R)}$ in 17 cases and $Pyrost^{(R)}$ in 3 cases. The average durations when bony union was achieved in radiograph were 13.8 weeks in whole cases, 12.5 weeks(range : 8 - 24 weeks) in $Lubboc^{(R)}$ graft cases and 15.7 weeks(range : 6 - 24 weeks) in $Surgibone^{(R)}$ graft cases. The tumor recurred in 4 cases, 1 case was recurred giant cell tumor at distal femur treated with mixed auto and $Lubboc^{(R)}$ graft and 2 cases were large cystic lesions at the proximal humerus diagnosed as simple bone cyst and at distal tibia diagnosed as fibrous dysplasia treated with $Surgibone^{(R)}$ graft and 1 case was aneurysmal bone cyst of the proximal tibia treated with $Lubboc^{(R)}$ graft. Wound infection occurred in 1 case. More transfusion was done in the cases that the lesion was larger than 5cm, the lesions were in the ilium or femur and the cases that were treated with mixed with autograft. This study implies that benign bone tumor is successfully treqated with curettage and xenograft or xenograft mixed with autograft. And also this method will reduce morbidity of donor site, intraoperative bleeding and post-transfusion complications.

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DENTAL MANAGEMENT OF CEMENTO-OSSIFYING FIBROMA: A CASE REPORT (백악질 골화성 섬유종의 치과적 접근 : 증례보고)

  • Han, Ji-Hye;Baik, Byeong-Ju;Yang, Yeon-Mi;Lee, Sun-Young;Kim, Jae-Gon
    • Journal of the korean academy of Pediatric Dentistry
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    • v.32 no.2
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    • pp.200-206
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    • 2005
  • Cemento-ossifying fibroma of the jaws is well circumscribed, generally slow-growing, benign lesions which enlarge in an expansile manner. Clinically it presents as a slowly enlarging lesion commonly in the premolar-molar area of the mandible and only occasionally in the maxilla and other locations. It occurs twice as often in females and primarily in the 20 to 30 year age group. Differential diagnosis should be peformed, preferably with other fibro-osseous lesions such as fibrous dysplasia. A faster growing and more destructive variant of cemento-ossifying fibroma sometimes occurs in patients under age 15 and is termed juvenile (aggressive) ossifying fibroma. Treatment is surgical removal with the extent depending on the size and location of the individual lesion. Recurrence is considered rare. A case involving a 12-year-old male patient with delayed eruption of right mandibular canine is discussed. Following an incisional biopsy, the histopathologic diagnosis established was cemento-ossifying fibroma. After the surgical enucleation of the lesion, no sign of recurrence was detected.

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