Craniomaxillofacial Fibrous Dysplasia Research Articles

Craniomaxillofacial Fibrous Dysplasia Improved Cosmetic and Occlusal Problem by Comprehensive Treatment: A Case Report and Review of Current Treatments

Fibrous dysplasia (FD) is a fibrous lesion of immature bone, with an incidence of 10–20% in the head and neck region. Most cases are monostotic, but when a lesion occurs on the maxillofacial region and spreads to the surrounding bone, it is classified as polyostotic, despite its localized occurrence. In some cases, surgical intervention is required to improve the cosmetic or functional disturbance of a FD in the maxillofacial region, but it is necessary to confirm symmetry of the maxillofacial region in real time, and a surgical support system is required to compensate. Furthermore, prosthetic intervention is considered when postoperative acquired defects occur or further cosmetic or occlusal function improvement is needed. A comprehensive approach by an oral surgeon and a maxillofacial prosthodontist is necessary for the successful treatment and rehabilitation of such patients. In this article, we describe the case of a craniomaxillofacial FD patient with facial asymmetry and denture incompatibility with improved quality of life measures by integrating surgical treatment using a navigation system and postoperative prosthetic rehabilitation. We also discuss recent diagnostic methods and treatment strategies for craniomaxillofacial FD in the literature.

Use of augmented reality navigation to optimise the surgical management of craniofacial fibrous dysplasia

The aim of this study was to apply an augmented reality (AR) navigation technique based on a head- mounted display in the treatment of craniofacial fibrous dysplasia and to explore the feasibility and the value of AR in craniofacial surgery. With preoperative planning and three-dimensional simulation, the normal anatomical contours of the deformed area were recreated by superimposing the unaffected side on to the affected side. We completed the recontouring procedures in real time with the aid of an AR navigation system. The surgical outcome was assessed by superimposing the postoperative computed tomographic images on to the preoperative virtual plan. The preparation and operation times were recorded. With intraoperative AR guidance, facial bone recontouring was performed uneventfully in all cases. The mean (SD) discrepancy between the actual surgical reduction and preoperative planning was 1.036 (0.081) mm (range: 0.913 (0.496) to 1.165 (0.498) mm). The operation time ranged from 50 to 80 minutes, with an average of 66.4 minutes. The preoperative preparation time ranged from 26 to 36 minutes, with a mean of 29.6 minutes. AR navigation–assisted facial bone recontouring is a valuable treatment modality in managing craniomaxillofacial fibrous dysplasia and shows benefits in improving the efficiency and safety of this complicated procedure.

POLYOSTOTIC CRANIOMAXILLOFACIAL FIBROUS DYSPLASIA ASSOCIATED WITH ACROMEGALY: A CASE REPORT

McCune-Albright syndrome (MAS) is a complex congenital disorder due to mutations at the onset of embryonic activation of postzygotic somatic cells in the GNAS1 gene. The phenotype is heterogeneous and includes polyostotic/monostotic fibrous dysplasia, cafe-au-lait skin spots, and hyperfunctioning endocrinopathies including excess of growth hormone (GH). Similarly, acromegaly as a manifestation of endocrine hyperfunction is uncommon and affects about 20% of patients with MAS. Here, we describe a 31-year-old woman with severe facial involvement of polyostotic fibrous dysplasia, in association with acromegaly and McCune-Albright syndrome, in which it was satisfactorily managed by surgical recontouring, with no clinical signs of relapse at a 12-year follow-up. Patients with craniomaxillofacial fibrous dysplasia associated with acromegaly may present significant facial asymmetries, which can be satisfactorily solved by cosmetic treatment, especially in patients with psychological problems and problems of severe social acceptance.

Prosthetic treatment of adolescents with craniomaxillofacial fibrous dysplasia

Craniomaxillofacial fibrous dysplasia may cause structural anomalies in the facial part of the skull, leading to anatomical and functional abnormalities in the stomatognathic system, requiring multi-disciplinary treatment. Depending on the location of dysplastic lesions, stage and course of the disease, each patient presents with a specific picture of the disorder and requires an individual therapeutic approach. Prosthetic treatment of patients at developmental age requires regular check-ups and frequent corrections or replacement of prosthetic restorations. This is particularly important in patients whose prosthetic field is deformed in such disorders as fibrous dysplasia or following numerous surgical procedures. The effectiveness of therapeutic procedures, which in such cases must be multidisciplinary, depends mainly on the commitment and cooperation of the patient and their carers. We present difficulties in the prosthetic rehabilitation of maxillofacial dysplasia based on the example of a patient diagnosed with fibrous dysplasia, severe oligodontia multiorgan congenital malformations, and a history of numerous surgical procedures within the stomatognathic system.

First two cases of craniomaxillofacial fibrous dysplasia from Nepal - case series.

Fibrous dysplasia is a benign fibro-osseous disease of the bone, which is most commonly associated with congenital mutations in cAMP regulating protein Gsα coded by GNAS-1 gene. Often it is seen involving the craniofacial skeleton and can range from an asymptomatic monostotic form to polyostotic variant involving almost all the bones of the skull, thereby leading to functional and esthetic problems. This requires a continuous monitoring of the involved region throughout the life of the patient, even after the surgical interventions. We are presenting two cases of craniofacial form of fibrous dysplasia. One case shows monostotic form, while the other case shows features of polyostotic form of disease. To the best of our knowledge, these are the first two cases of craniomaxillofacial fibrous dysplasia from Nepal, which will be reported and published.

네비게이션 및 내시경을 사용한 악안면 섬유성 이형성증의 안면부 축소술 1예

네비게이션 및 내시경을 사용한 악안면 섬유성 이형성증의 안면부 축소술 1예

Treatment of Craniomaxillofacial Fibrous Dysplasia

Fibrous dysplasia is a benign osseous disease, although radical resection is commonly recommended because of the possibility of recurrence or malignant change. However, radical resection is undesirable, particularly in the maxillofacial region, because facial bones are difficult to replace in an aesthetically-pleasing manner after radical excision. In our department, conservative shaving, as reported by Chen and Noordhoff, is used to reduce tumor volume while maintaining facial aesthetics that are as normal as possible. We have experienced eight cases of craniomaxillofacial fibrous dysplasia and successfully applied conservative shaving to the maxillofacial lesions. These cases have exhibited no recurrence, and their facial contours have improved remarkably.

Clinical characteristics of craniomaxillofacial fibrous dysplasia

BackgroundThe clinical characteristics of craniomaxillofacial fibrous dysplasia (FD) have not been clearly identified. The objective of this meta-analysis is to assess the predominance of the monostotic form of FD using an evidence-based review. Furthermore, we examined the laterality and sex dominance of FD in patients from international study populations. MethodsWe performed a systematic search of PubMed, Embase, Cochrane Central Register of Systematic Reviews, Cochrane Central Register of Controlled Trials and EBSCO for trials published through August 2013. Data extracted from the literature were analysed with Review manager 5.0.24. ResultsThe results of this study showed that unilateral FD occurred more frequently than bilateral FD (RR, 12.37; 95% CI, 2.92–61.24; P = 0.008, N = 263 patients). For unilateral FD, there was no significant difference between cases involving the left or right side of the face (RR, 0.98; 95% CI, 0.66–1.44; P = 0.91; N = 201 patients). There were no significant sex-dependent differences for monostotic and polyostotic forms of craniomaxillofacial FD. ConclusionThere is a significantly higher percentage of the unilateral form than the bilateral form in the craniomaxillofacial FD studies analysed, and an almost equal distribution of left- and right-sided unilateral FD. These proportions were maintained among males and females and there were nearly equal frequencies of monostotic and polyostotic FD.

Spontaneous osteosarcoma in craniomaxillofacial fibrous dysplasia: clinical and computed tomographic features in 8 cases.

The aim of this study was to evaluate the patient demographic and computed tomography (CT) imaging findings of spontaneous osteosarcoma in craniomaxillofacial fibrous dysplasia. Ten cases of spontaneous osteosarcoma in craniomaxillofacial fibrous dysplasia diagnosed during 1993-2013 were reviewed. Eight cases with CT images were reviewed. The mean age of diagnosis of osteosarcoma was 37.8 years (range, 8-55 years). The presence of a soft tissue extension component beyond the area of cortical bone destruction was demonstrated radiographically in 7 out of 8 cases. Mineralization of tumor matrix was seen in 6 cases. Periosteal reaction was demonstrated in only 1 case; 1 out of 6 cases showed significant enhancement on postcontrast CT images. Nine patients had surgical resections of tumor. Seven of them died of tumor, with a mean survival time of 5.3 years. This study provides CT imaging features with clinical information of spontaneous osteosarcoma in craniomaxillofacial fibrous dysplasia.

Surgical Treatment of Craniomaxillofacial Fibrous Dysplasia

Fibrous dysplasia (FD) is a tumor-like growth that consists of replacement of the medullary bone with fibrous tissue, causing the expansion and weakening of the areas of bone involved. The most commonly affected bones are facial bones, causing a number of facial cosmetic and functional problems. From December 2008 to July 2012, 10 patients with craniomaxillofacial fibrous dysplasia were treated by conservative resection and local recontouring. The patients were followed up yearly, with an average of 3 years; the longest follow-up period was 5 years. All the 10 patients received appropriate treatment and histopathological examinations were performed to confirm the diagnosis of FD. Four patients with zygoma involved had received partial zygoma osteoectomy and 2 patients received mandibular partial osteoectomy. Average time of follow-up was 3 years, with a range from 1 to 5 years, and all patients obtained satisfactory aesthetic and functional results. In most patients, a conservative surgery will achieve good functional and aesthetic results. For patients with mild symptoms, the aesthetic effect should be given priority while for the heavier patients the restoration of function and aesthetic effects should all be taken into account.

Spontaneous Malignant Transformation in Craniomaxillofacial Fibrous Dysplasia

Spontaneous malignant transformation in craniomaxillofacial fibrous dysplasia (FD) is extremely rare and its clinicopathological characteristics remain largely unknown. Here, we aimed to characterize the epidemiology and clinicopathological features of malignancies arising from preexisting FD by presenting data from a Chinese tertiary referral hospital and review of English and Chinese literatures. The craniomaxillofacial disease registry of Shanghai Ninth People's Hospital was searched and reviewed to collect relevant information for patients diagnosed as malignant transformation in craniomaxillofacial FD between January 1993 and December 2010. An English and Chinese literature review was conducted to retrieve pertinent cases published in the past 2 decades with preset inclusion criteria. All included cases were further analyzed with regard to associated clinical and pathological variables. Three cases with osteosarcoma arising from previous craniomaxillofacial FD were found at our institution and 35 other cases were identified by literature review. These uncommon entities usually occurred in adults with a mean age of 39.8 years and equal gender preponderance. Maxilla remained the most common sites for malignancies followed by mandible and zygoma. Most malignancies were diagnosed as osteosarcoma followed by fibrosarcoma, chondrosarcoma, and malignant fibrous histiocytoma. Radical resection alone or with postoperative radiotherapy/chemotherapy remains the main treatment strategy with unfavorable prognosis due to local recurrence and distant metastasis. Taken together, our findings might for the first time provide the comprehensive information regarding the epidemiology, clinicopathological features, treatment, and prognosis of malignancies in craniomaxillofacial FD. Further investigations are warranted to improve early diagnosis and proper treatment for these uncommon entities.

An epidemiological and clinical analysis of craniomaxillofacial fibrous dysplasia in a Chinese population

BackgroundCraniomaxillofacial fibrous dysplasia (FD) is a benign bone lesion characterized by facial disfigurement and functional impairment. The aim of this study was to characterize the epidemiological and clinical features of craniomaxillofacial FD by presenting data from a representative Chinese population during a 15-year period (1994–2009).MethodThe craniomaxillofacial disease registries of two Chinese tertiary referral hospitals (Shanghai Ninth People’s Hospital and Stomatological hospital of Jiangsu Province) were searched and reviewed to collect relevant information for patients with craniomaxillofacial FD between Jan.1994 and Dec.2009. All included cases were further analyzed with regard to associated epidemiological and clinicopathological variables.ResultsA total number of 266 cases with definitive diagnosis were identified with 219 primary cases and 47 recurrent cases. There were 111 males and 155 females with a male to female ratio of 0.716:1. They were clinically categorized into three groups: monostotic (71.1%), polysotic (27.4%) and Albright syndrome (1.5%). Maxilla alone or with adjacent bones was the most common affected site. The serum alkaline phosphatase (ALP) in patients was much higher than that in healthy control, whereas comparable between primary patients and recurrent ones. Three patients (3/266, 1.1%) with polysotic lesions underwent spontaneous malignant transformation into osteosarcoma. The majority of patients underwent conservative surgery, while the others received radical resection with or without reconstruction.ConclusionsCraniomaxillofacial FD is a rare bony disorder with defined epidemiological and clinicopathological features in Chinese population. Further investigations are warranted to establish the optimized timing, treatment strategy and prognostic prediction for this clinical entity.

Craniofacial fibrous dysplasia (CFD) of the maxilla in an 11-year old boy: A case report

We present the case of a surgically treated 11-year old boy with a diagnosis of craniomaxillofacial fibrous dysplasia (CFD) in the maxillary sinus. When first seen in the outpatient clinic of our department he had minimal symptoms. After initial radiological diagnostics by computed tomography scans (CT-scans) the patient was treated operatively by radical excision of the tumor. The radiographs showed the typical intramedullary located and well-defined lesions, which eroded the cortical bone with the typical appearance of fibrous dysplasia. The histopathology showed the typical curved extending fibrous trabeculae in C, O and Y-shape which were embedded in a moderately cellular morphologically inconspicuous stroma, confirming the initial suspicion of fibrous dysplasia of the maxillary bone. Cone beam tomography was a valuable tool in determining the re-ossification of bone at the affected side. Local resection can be curative in limited disease.

Image-Guided Navigation in Optimizing Surgical Management of Craniomaxillofacial Fibrous Dysplasia

Fibrous dysplasia is a benign developmental dysplastic disorder of the bone in which abnormal fibroblastic proliferation replaces the normal bone matrix. Craniomaxillofacial fibrous dysplasia can cause severe deformity with devastating functional and aesthetic consequence. Precise surgical removal of the fibro-osseous tissue is the key to ultimately restore normal function and aesthetics. Navigational surgical tool has become a useful adjunct in the surgical management of the craniomaxillofacial skeleton. Thirteen patients with facial asymmetry and deformity caused by craniomaxillofacial fibrous dysplasia were enrolled into this study. With preoperative planning and three-dimensional simulation, normal anatomic contours of the deformed area were recreated by superimposing unaffected to the affected side. After registration, surgical facial recontouring was performed under the guidance of navigation system. Good accuracy was achieved with the registration of patient's facial skeleton and preoperative three-dimensional reconstructed model in all the cases. With intraoperative navigational guidance, facial bone recontouring was performed uneventfully in all cases. Surgical accuracy was evaluated by comparing the preoperative and postoperative computed tomography scan measurement. The mean maximum discrepancy between the actual surgical reduction and preoperative planning was less than 2 mm. Symptoms associated with optical nerve compression were eliminated in affected individuals. All patients' facial symmetry and aesthetics were improved. Navigation-guided facial bone recontouring is a valuable treatment modality in managing craniomaxillofacial fibrous dysplasia.

P.160 Treatment of craniomaxillofacial fibrous dysplasia with pamidronate – Preliminary report

Fibrous dysplasia is a benign fibro-osseous disease of bone of unknown etiology. Its occurrence in the craniomaxillofacial skeleton is frequent and varies in severity from an asymptomatic monostotic lesion to polyostotic involvement resulting in progressive functional deficit and aesthetic problems.With the advent of refined instrumentation and craniofacial surgical techniques, a more aggressive, non-disabling approach to these benign yet deforming fibro-osseous growths is possible. In some patients, complete excision of the involved bone with graft reconstruction of the resultant defect with primary autogenous bone may be possible. Lifelong continuous ongoing monitoring of the involved region is required throughout the patient's life.

Orthognathic surgery in craniomaxillofacial fibrous dysplasia.

In craniomaxillofacial fibrous dysplasia, jaw involvement often causes facial asymmetry, an occlusal cant, and loss of teeth. Although conservative management of fibrous dysplasia affecting the jaws is widely practiced, orthognathic surgery is indicated in such cases to restore occlusion and correct dentofacial deformity brought on by the disease process. Since 1981, the Craniofacial Center at Chang Gung Memorial Hospital in Taiwan has treated a total of 84 patients with craniomaxillofacial fibrous dysplasia. Of these, 55 (65%) had fibrous dysplasia affecting the jaws (Zone 4). Between 1988 and 1997, orthognathic surgery was performed on 1 male and 4 female patients with fibrous dysplasia involving the teeth-bearing jaws. One patient had localized fibrous dysplasia that involved the mandible. The other 4 patients had polyostotic craniofacial involvement of Zones 1, 2, or 3 and 4A. The patient with isolated mandibular involvement and 2 patients with maxillary fibrous dysplasia had single-jaw surgery. The other 2 patients with maxillary involvement required simultaneous two-jaw surgery to correct the dentofacial deformities resulting from the disease process. Follow-up ranged from 12 months to 9 years. All the patients had stable occlusion, good facial aesthetics, and no further recurrence after surgery. The long-term stability of the achieved occlusion and facial appearance confirms that adequate healing in fibrodysplastic bone is to be expected using the standard fixation.

Treatment of Craniomaxillofacial Fibrous Dysplasia

Twenty-eight craniomaxillofacial fibrous dysplasia patients were treated as early as the symptoms occurred. The principles of surgical treatment were based on the zones of involvement: total excision of dysplastic bone of fronto-orbital, zygoma, and upper maxillary origin (zone 1) and bone reconstruction primarily; conservative excision on hair-bearing skull (zone 2), central cranial base (zone 3), and tooth-bearing bones (zone 4); and optic canal decompression on patients with orbital dysplasia and decreasing visual acuity. Patients were followed for 1 to 11 years (average 5.3 years). No recurrence or invasion of the fibrous dysplasia into the grafted bone was seen. One patient had orthognathic maxillary osteotomy on the reconstructed maxilla 6 years after initial reconstruction. Five of 19 patients with alveolar dysplasia had a recurrence and were reshaped. One patient had mandibular sagittal osteotomies to set back the prognathic, fibrous dysplasic mandible after three attempts at conservative shaving. Another patient with mandibular fibrous dysplasia had recurrence with pain and a hemimandibulectomy with successful immediate free vascularized iliac bone graft reconstruction.