Cherubism: best clinical practice

Cherubism is a skeletal dysplasia characterized by bilateral and symmetric fibro-osseous lesions limited to the mandible and maxilla. In most patients, cherubism is due to dominant mutations in the SH3BP2 gene on chromosome 4p16.3. Affected children appear normal at birth. Swelling of the jaws usually appears between 2 and 7 years of age, after which, lesions proliferate and increase in size until puberty. The lesions subsequently begin to regress, fill with bone and remodel until age 30, when they are frequently not detectable

Management of Orofacial Manifestations of Juvenile Idiopathic Arthritis: Interdisciplinary Consensus-Based Recommendations

Involvement of the temporomandibular joint (TMJ) is common in juvenile idiopathic arthritis (JIA). TMJ arthritis can lead to orofacial symptoms, orofacial dysfunction, and dentofacial deformity with negative impact on quality of life. Management involves interdisciplinary collaboration. No current recommendations exist to guide clinical management. We undertook this study to develop consensus-based interdisciplinary recommendations for management of orofacial manifestations of JIA, and to create a future research agenda related to management of TMJ arthritis in children with JIA. Recommendations were developed using online surveying of relevant stakeholders, systematic literature review, evidence-informed generation of recommendations during 2 consensus meetings, and Delphi study iterations involving external experts. The process included disciplines involved in the care of orofacial manifestations of JIA: pediatric rheumatology, radiology, orthodontics, oral and maxillofacial surgery, orofacial pain specialists, and pediatric dentistry. Recommendations were accepted if agreement was >80% during a final Delphi study. Three overarching management principles and 12 recommendations for interdisciplinary management of orofacial manifestations of JIA were outlined. The 12 recommendations pertained to diagnosis (n = 4), treatment of TMJ arthritis (active TMJ inflammation) (n = 2), treatment of TMJ dysfunction and symptoms (n = 3), treatment of arthritis-related dentofacial deformity (n = 2), and other aspects related to JIA (n = 1). Additionally, a future interdisciplinary research agenda was developed. These are the first interdisciplinary recommendations to guide clinical management of TMJ JIA. The 3 overarching principles and 12 recommendations fill an important gap in current clinical practice. They emphasize the importance of an interdisciplinary approach to diagnosis and management of orofacial manifestations of JIA

Pediatric oral and Maxillofacial Surgery

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Minimally invasive maxillofacial surgery / Maria J. Troulis, DDS, MSc, Associate Professor of Oral and Maxillofacial Surgery, Director, Residency Training Program in OMFS, Harvard School of Dental Medicine, Massachusetts General Hospital, Boston, Massachusetts, Leonard B. Kaban, DMD, MD, Department Head, and Walter C. Guralnick, Professor of Oral and Maxillofacial Surgery, Harvard School of Dental Medicine, Massachusetts General Hospital, Boston. Massachusetts.

dental bookfair2015Includes bibliographical references and index.xii, 240 pages

Changes in the condyle and disc in response to distraction osteogenesis of the minipig mandible

Purpose: Distraction osteogenesis (DO) is a commonly used technique for mandibular lengthening, but changes in the temporomandibular joint have not been well documented. The purpose of this study was to evaluate the effect of DO, at varying rates, on the mandibular condyle and articular disc. Materials and Methods: Semiburied distractors were placed via submandibular incisions in 15 minipigs. Two unoperated animals served as controls. The protocol consisted of 0 day latency and rates of 1, 2, or 4 mm/d for a 12-mm gap. After the animals were killed (0, 24, or 90 days), ipsilateral and contralateral condyles and discs were harvested and evaluated to determine changes in 1) condylar form and size, 2) condylar surface, and 3) the articular disc. Results: Articular surfaces of the condyles in control animals were smooth, with no irregularities or erosions. In animals undergoing distraction, ipsilateral condyles showed increasing changes in morphology and AP dimension, and surface contour irregularities as the DO rate increased. These changes were present, but to a lesser degree, in the contralateral condyles. Articular discs of both ipsilateral and contralateral sides showed variable thinning at the medial aspect at end DO. After 90 days, changes in the condyles and discs were reduced by remodeling except in the 4 mm/d DO groups. Conclusions: Results of this preliminary study indicate that gross changes occur in condyles and discs after unilateral mandibular DO. These changes are more severe at faster distraction rates (4 mm/d) and tend to resolve during neutral fixation when a rate of 1 mm/d is used. © 2002 American Association of Oral and Maxillofacial Surgeons J Oral Maxillofac Surg 60:1327-1333, 200

Morbidity and mortality rates after maxillomandibular advancement for treatment of obstructive sleep apnea

Purpose: To compare morbidity and mortality rates in obstructive sleep apnea (OSA) versus dentofacial deformity (DFD) patients undergoing equivalent maxillofacial surgical procedures. Patients and Methods: Patients with OSA who underwent maxillomandibular advancement with genial tubercle advancement in the Massachusetts General Hospital Department of Oral and Maxillofacial Surgery from December 2002 to June 2011 were matched to patients with DFD undergoing similar maxillofacial procedures during the same period. They were compared regarding demographic variables, medical comorbidities, perioperative management, intraoperative complications, early and late postoperative complications, and mortality rate. Results: A study group of 28 patients with OSA and a control group of 26 patients with DFD were compared. The patients with OSA were older (41.9 +/- 12.5 years vs 21.7 +/- 8.6 years), had a higher American Society of Anesthesiologists classification (2.0 +/- 0.5 vs 1.3 +/- 0.6), and had a higher body mass index (29.6 +/- 4.7 kg/m(2) vs 23.0 +/- 3.1 kg/m(2)). They also had a greater number of medical comorbidities (2.4 +/- 2.3 vs 0.7 +/- 1.0). More OSA patients than DFD patients had complications (28 [100%] vs 19 [73%], P = .003), and the total number of complications in the OSA group was higher (108 vs 33, P < .001). Of the complications, 13.9% in the OSA group and 3.0% in the DFD group were classified as major. The absolute risk of a complication was 3.9 for the OSA group versus 1.3 for the DFD group. The relative risk of complications in OSA patients compared with DFD patients was 3.0. No difference in mortality rate was found. Conclusions: The patients in the OSA group were older, had more comorbidities, and ultimately had a greater number of early, late, minor, and major complications than those in the DFD group. The incidence of death in both groups was zero. Maxillomandibular advancement appears to be a safe procedure regarding mortality rate, but OSA patients should be counseled preoperatively regarding the relative increased risk of complications. (C) 2016 American Association of Oral and Maxillofacial Surgeons741020332043COORDENAÇÃO DE APERFEIÇOAMENTO DE PESSOAL DE NÍVEL SUPERIOR - CAPESsem informaçã

Antiangiogenic therapy with interferon alpha for giant cell lesions of the jaws

Purpose: Giant cell tumors are classified and treated based on their biologic behavior. We hypothesize that they are proliferative vascular lesions and would be expected to respond to antiangiogenic therapy. The purpose of this report is to present a treatment protocol consisting of enucleation, with preservation of vital structures, followed by subcutaneous interferon alpha. Materials and Methods: Patients with a biopsy-confirmed giant cell lesion satisfying criteria for “aggressive giant cell tumor” were included. Instead of wide en bloc resection, lesions were enucleated and the patients started on interferon alpha-2 or beta (3,000,000 units/m2) 48 to 72 hours postoperatively. The subjects were followed by clinical examination and radiography, immediately after surgery and every 3 months until the bone cavity completely healed. Thereafter, follow-up was every 6 months. Results: Eight patients (7 females), with a mean age of 18.7 ± 11.1 years, have been enrolled. Six tumors were in the posterior mandible, and 2 were in the anterior maxilla. The mean size was 29.0 mm (range, 15 to 70 mm). All patients underwent enucleation. There were no postoperative complications, and all patients tolerated interferon. There was no evidence of tumor growth during treatment. Seven of 8 patients have completed interferon therapy, and there have been no recurrences during 1 to 6 years of follow-up. The other patient continues on treatment with no evidence of disease. Conclusion: Antiangiogenic therapy, in combination with curettage, is a promising strategy for treatment of aggressive giant cell tumors. Combined treatment results in a high rate of tumor control with decreased operative morbidity compared with conventional treatment. © 2002 American Association of Oral and Maxillofacial Surgeons J Oral Maxillofac Surg 60:1103-1111, 200

Immune Surveillance Plays a Role in Locally Aggressive Giant Cell Lesions of Bone

BackgroundGiant cell lesions are locally aggressive intraosseous neoplasms with capacity to metastasize. The role of immune surveillance in the pathophysiology of giant cell lesions is poorly understood, and understanding what role the immune system plays in giant cell lesions may lead to the development of more effective treatment. The aim of this study was to explore the role of immune surveillance in giant cell lesions by examining the expression of the HLA class I and class II antigens and tumor infiltrating lymphocytes. In addition, we examined the role of the immune modulating surface antigen B7-H3, which belongs to the B7 superfamily, a group of molecules that modulates T-cell responses.Questions/purposes(1) Is an immune response elicited by giant cell lesions? (2) Do clinically relevant human leukocyte antigen (HLA) defects exist in giant cell lesions? (3) Is B7-H3 a clinically relevant immune modulator?MethodsThe study sample was derived from the population of patients presenting to the Massachusetts General Hospital for evaluation and management of giant cell lesions from 1993 to 2008. We included patients with histologically confirmed giant cell lesions with a minimum followup of 6&nbsp;months. Patients with systemic diseases (n = 4 [3%]), syndromes associated with giant cell lesions (n = 4 [3%]), and those without sufficient followup (n = 26 [19%]), inadequate records (n = 7 [5%]), or inadequate tissue available (n = 2 [1%]) were excluded. Tissue microarray, containing 288 tissue cores for 93 patients, was carefully constructed. This contained tissue from 45 patients with maxillofacial lesions, 38 with aggressive and seven with nonaggressive lesions, and 48 patients with axial and appendicular lesions, 30 with aggressive lesions and 18 with nonaggressive lesions. The population mean age was 28 ± 12&nbsp;years and the duration of followup was 4 ± 3&nbsp;years. The tissue microarray was immunohistochemically stained with monoclonal antibodies specific for HLA classes I and II and B7-H3 antigens and analyzed for tumor infiltrating lymphocytes. Antigen expression was examined in multinucleated giant cells and mononuclear stromal cells. The results were correlated with local invasion and tumor aggressiveness, which is based on accepted staging criteria.ResultsTumor infiltrating lymphocytes were detected in all the tumors. The mean number of CD8+ T cell infiltration was lower in aggressive tumors (median, 4.8; interquartile range [IQR], 0.4-13.4), when compared with nonaggressive tumors (median, 15.8; IQR, 4.3-46.3; p = 0.007). HLA class I antigens were highly expressed by multinucleated giant cells in all tumors, but were lightly expressed on mononuclear stromal cells in 53% (45 of 84) to 73% (56 of 77) of tumors. HLA class I antigen low expression in mononuclear stromal cells was associated with tumor aggressiveness (odds ratio [OR], 4.3; p = 0.005). Low HLA class I expression combined with low CD8+ T cell infiltration was most highly associated with tumor aggressiveness (OR, 7.81; p = 0.011). B7-H3 antigen was expressed in 36.9% mononuclear stroma cells and also was associated with local tumor invasion (OR, 1.36; p &lt; 0.001). Similarly, giant cell lesions with high B7-H3 expression and low CD8+ tumor infiltrating lymphocytes were associated with increased tumor aggressiveness (OR, 8.89; p = 0.0491).ConclusionsLocally aggressive giant cell lesions are associated with low HLA class 1 antigen expression, low CD8+T cell infiltration, and high expression of the immune modulator B7-H3.Clinical relevanceFailure of immune surveillance implies that there may be an opportunity to target aspects of the immune surveillance machinery to treat giant cell lesions

Immune Surveillance Plays a Role in Locally Aggressive Giant Cell Lesions of Bone

Giant cell lesions are locally aggressive intraosseous neoplasms with capacity to metastasize. The role of immune surveillance in the pathophysiology of giant cell lesions is poorly understood, and understanding what role the immune system plays in giant cell lesions may lead to the development of more effective treatment. The aim of this study was to explore the role of immune surveillance in giant cell lesions by examining the expression of the HLA class I and class II antigens and tumor infiltrating lymphocytes. In addition, we examined the role of the immune modulating surface antigen B7-H3, which belongs to the B7 superfamily, a group of molecules that modulates T-cell responses. (1) Is an immune response elicited by giant cell lesions? (2) Do clinically relevant human leukocyte antigen (HLA) defects exist in giant cell lesions? (3) Is B7-H3 a clinically relevant immune modulator? The study sample was derived from the population of patients presenting to the Massachusetts General Hospital for evaluation and management of giant cell lesions from 1993 to 2008. We included patients with histologically confirmed giant cell lesions with a minimum followup of 6 months. Patients with systemic diseases (n = 4 [3%]), syndromes associated with giant cell lesions (n = 4 [3%]), and those without sufficient followup (n = 26 [19%]), inadequate records (n = 7 [5%]), or inadequate tissue available (n = 2 [1%]) were excluded. Tissue microarray, containing 288 tissue cores for 93 patients, was carefully constructed. This contained tissue from 45 patients with maxillofacial lesions, 38 with aggressive and seven with nonaggressive lesions, and 48 patients with axial and appendicular lesions, 30 with aggressive lesions and 18 with nonaggressive lesions. The population mean age was 28 ± 12 years and the duration of followup was 4 ± 3 years. The tissue microarray was immunohistochemically stained with monoclonal antibodies specific for HLA classes I and II and B7-H3 antigens and analyzed for tumor infiltrating lymphocytes. Antigen expression was examined in multinucleated giant cells and mononuclear stromal cells. The results were correlated with local invasion and tumor aggressiveness, which is based on accepted staging criteria. Tumor infiltrating lymphocytes were detected in all the tumors. The mean number of CD8+ T cell infiltration was lower in aggressive tumors (median, 4.8; interquartile range [IQR], 0.4-13.4), when compared with nonaggressive tumors (median, 15.8; IQR, 4.3-46.3; p = 0.007). HLA class I antigens were highly expressed by multinucleated giant cells in all tumors, but were lightly expressed on mononuclear stromal cells in 53% (45 of 84) to 73% (56 of 77) of tumors. HLA class I antigen low expression in mononuclear stromal cells was associated with tumor aggressiveness (odds ratio [OR], 4.3; p = 0.005). Low HLA class I expression combined with low CD8+ T cell infiltration was most highly associated with tumor aggressiveness (OR, 7.81; p = 0.011). B7-H3 antigen was expressed in 36.9% mononuclear stroma cells and also was associated with local tumor invasion (OR, 1.36; p < 0.001). Similarly, giant cell lesions with high B7-H3 expression and low CD8+ tumor infiltrating lymphocytes were associated with increased tumor aggressiveness (OR, 8.89; p = 0.0491). Locally aggressive giant cell lesions are associated with low HLA class 1 antigen expression, low CD8+T cell infiltration, and high expression of the immune modulator B7-H3. Failure of immune surveillance implies that there may be an opportunity to target aspects of the immune surveillance machinery to treat giant cell lesions