The xrays showed typical bone lesions in the infraorbital region of the left maxillary bone without involvement of the orbit. Fibrous dysplasia polyostotic free download as powerpoint presentation. Fibrous dysplasia not uncommonly involves the skull and facial bones, usually. The ximage classification of fibrous dysplasia showed that there were 44 cases of ground glass 61. Fibrous dysplasia free download as powerpoint presentation. This study evaluates the possibility of diagnosing fibrous dysplasia on imaging alone, without biopsy of the lesion, which is often burdensome for the patient.
Fibrous dysplasia of bone is a rare sporadic benign congenital condition in which. The patient had previously received treatment from several general dentists and several specialists, without accurate diagnosis. The involvement of the craniofacial skeleton is not. Generally, the occurrence rate is higher in the maxilla than in the mandible. Fibrous dysplasia is a congenital present at birth condition that affects bone growth and development. However, localized fibrous dysplasia on mr imaging often mimics a tumor because fibrous tissue can enhance brilliantly after the injection of contrast material. The aim of this case report is to discuss the orthodontic treatment of a yearold patient with fibrous dysplasia in the left maxilla.
We describe one case of maxillary bone fibrous dysplasia that, in spite of its benign nature, caused facial deformity. Surgery is an effective management option and involves removing the diseased bone via an intraoral approach. In mfd, the maxilla is more commonly affected than the mandible. Oral rehabilitation with dental implants for teeth.
Pdf craniofacial fibrous dysplasia cfd of the maxilla. Clinical, radiological, and histological problems arise relating to fibrous dysplasia of the maxilla. The histological diagnosis may be very difficult, since any tumoural, reactive, or healing process may simulate fibrous dysplasia. Intraoral microvascular anastomosis of an iliac free flap. Fibrous dysplasia ossifying fibroma of the maxilla and mandible. Midfacial degloving approach for management of the maxillary. Fibrous dysplasialike appearance of the frontal process of. A 22 yrs female presented with swelling of left facial region for the last 5yrs with nasal obstruction for the last 2yrs. Maxillary osteosarcoma with fibrous dysplasia of frontal. A case of fibrous displasia of ethmoido maxillary complex is presented. Instead of maturing into solid bone, affected bones stay at the immature fibrous stage so are weak and misshapen. It affects the maxilla more frequently than the mandible and may involve one monostotic or less. Monostotic fibrous dysplasia involving the mandible. Aggressive fibrous dysplasia of the maxillary sinus.
Fibrous dysplasia and ossifying fibroma of the paranasal. A retrospective study of consecutive paranasal sinus ct scans was preformed on outpatients without a history of trauma or previous surgery in our hospital during 6 months. Conventional radiographic findings reveal characteristics of fibrous dysplasia. The results of esthetic surgery to the nasal pyramid were satisfactory. Histologically its a fibroosseous lesion characterised by the replacement of normal bone with a variable amount of fibrous tissue and woven bone which causes progressive expansion and deformity of bones. Fibrous dysplasia is a rare bone disorder characterized by the replacement. Fibrous dysplasia, maxilla, monostotic, polyostotic. Double calvarial fibrous dysplasia manish kumar kasliwal, ashok k. Fibrous dysplasia fd is a benign bone lesion characterized by the progressive replacement of normal bone with fibroosseous connective tissue. In fd, the spatial definition and structural distinction of cortical bone, cancellous bone, and bone marrow that is achieved through normal modeling is blurred and the distinct territories tend to become structurally continuous and homogeneous. Monostoticinvolve one bone polyostotic involve multiple bone. Features of malignancy include osteolysis, destruction of sclerotic margins, and cortical destruction with soft tissue extension. Fibrous displasia is a skeletal developmental disorder of the boneforming mesenchyme that manifests as a defect in osteoblastic differentiation and maturation.
The radiographic appearance of this lesion varies widely in the jaw. Fibrous dysplasia fd is a rare bone disease caused by postzygotic somatic activating mutations in the gnas gene, which lead to constitutive activation of adenylyl cyclase and elevated levels of cyclic amp, which act on downstream signaling pathways and cause normal bone to be replaced with fibrous tissue and abnormal woven bone. The clinical behavior and rapid progression of fd renders the treatment. The bony lesion shows a homogenous matrix with obliteration of maxillary sinus cavity. A 29yearold woman with a history of maxillary fibrous dysplasia presented to. Fibrous dysplasia is a benign fibrousosseous lesion in which normal bone is. Fibrous dysplasia mccune albright syndrome fdmas represents a wide spectrum of diseases. Mahapatra department of neurosurgery, neurosciences centre, all india institute of medical sciences, new delhi 110 029, india this pdf is available for free download from a site hosted by medknow publications. Fibrous dysplasia and ossifying fibroma of the paranasal sinuses volume 112 issue 10 d. Dec 01, 2003 fibrous dysplasia of the maxillary sinus can be characterized by facial soft tissue swelling on the affected side contributing to facial asymmetry and a drooping smile, underlying prominence of the malar eminence, ipsilateral enlargement of the maxillary vestibular bone and free gingiva, and a distinctive radiographic appearance. Multidisciplinary approach for management of a patient with. Fibrous dysplasia is a disorder of the bone where an individuals healthy bone tissue is replaced with fibrous or scarlike tissue. Fibrous dysplasia is a nonneoplastic hamartomatous developmental fibroosseous lesion of bone. The purpose of this study was to determine its prevalence and findings on ct.
The diagnosis of fibrous dysplasia on ct is usually straightforward 2, 3. Here, we are presenting two case reports of fd involving the maxilla. Although fd presents radiographic patterns, it has to be analyzed histologically for a final diagnosis. Craniofacial, fibrous dysplasia, monostotic, polyostotic. The maxilla is the most commonly affected area of facial bone, resulting in facial asymmetry and functional disorders. The next most common area of skull base involvement was the sphenoid bone 43%, followed by the frontal bone 33% figure 4 and maxilla 29%. Intramedullary curettage and threedimensional surface modelling of zigomaticomaxillary fibrous dysplasia. These weakened bones can experience fractures and or become deformed. Typical microscopic findings include irregular spindles of woven bone, usually nonmineralized, scattered throughout a fibrocellular matrix. Pdf frontoorbital sphenoethmoidal fibrous dysplasia. Endoscopic endonasal removal of a large fibrous dysplasia. Fibrous dysplasia is categorized as either monostotic or polyostotic and may occur as a component of mccunealbright syndrome or the rare mazabraud syndrome. Fibrous dysplasia fd, also known as lichtensteinjaffe disease, is a bone developmental anomaly due to a genetic defect in the osteoblastic differentiation caused by mutation in the gnas1 gene and characterized by a focal replacement of normal bone with fibrous stroma and islands of immature woven bone 1, 2. As a result, a fibrous dysplasia patient will have weaker bones than other individuals.
The purpose of this study was to report six cases of fibrous dysplasia associated with the maxillary sinus. Greco and steiner 1996 examined fibrous dysplasia cases and suggested that abnormal osteoblastic maturation of the bone forming mesenchyme has one of the most important alterations in this condition. An unusual complication possibly arising from the surgical. Intraoral microvascular anastomosis of an iliac free flap for maxillary fibrous dysplasia. Fibrous dysplasia fd is a benign bone lesion characterized by the. Maxillary lesions may extend to involve the maxillary sinus, zygoma, sphenoid bone and floor of the orbit and require surgical intervention resulting in an acquired defect of the. Orthodontic treatment of patient with maxillofacial.
A free powerpoint ppt presentation displayed as a flash slide show on id. Download fulltext pdf download fulltext pdf download fulltext. Intraoral microvascular anastomosis of an iliac free flap for. Craniofacial fibrous dysplasia is 1 of 3 types of fibrous dysplasia that can affect the bones of the craniofacial complex, including the mandible and maxilla.
Clinically, the monostotic form is characterized by a lytic expanding bone lesion, which may cause pain, deformity, fractures, and nerve entrapment. Fibrous dysplasia of ethmoidomaxillary complex springerlink. Jun 22, 2011 it is helpful in distinguishing fibrous dysplasia from a malignancy. With fibrous dysplasia, the bone grows in the form of a pain free swelling, while the maxilla and mandible are most frequently involved in the headneck zone, the ethmoid bone is infrequently involved. In this study, we examined radiographic patterns which. Yadan fan1, jiannan liu 1, chenping zhang 1, zhiyuan zhang 1, huawei yang2, jingzhou hu 1 1department of oral and maxillofacialhead and neck oncology, ninth peoples hospital, shanghai key laboratory of stomatology and shanghai research institute of stomatology, shanghai jiao tong university school of medicine. Recurrent monostotic fibrous dysplasia in the mandible hindawi. Fibrous dysplasia can present in myriad ways within the skull base. In this article we report the case of a patient with fibrous dysplasia occupying the entire left maxillary sinus with orbitary extension confirmed in the. This pictorial essay provides various imaging findings of fibrous dysplasia and examples of the underlying histopathology, gross intraoperative findings, and clinical characteristics.
Radiological investigation coupled with biopsy confirmed a diagnosis of fibrous dysplasia. Monostotic fibrous dysplasia is more common than the polyostotic form and usually involves jaw bones, ribs and femur. Clinical presentation may occur at any age, with the majority of lesions being detected by the age. Here we present a 16yearold female with a fibrous dysplasia in the maxilla and obliteration of the sinus in the same side. Double calvarial fibrous dysplasia bioline international.
Ppt fibrous dysplasia powerpoint presentation free to. Pdf craniofacial fibrous dysplasia cfd of the maxilla in. Pdf fibrous dysplasia of maxilla ijoart editor academia. Pdf endoscopic surgery for fibrous dysplasia of the. Fibrous dysplasia involves the maxilla almost twice as often as the. A, nineteen months after right partial maxillectomy for fibrous dysplasia, before reconstruction. Immediate reconstruction with free double costochondral. Dec 25, 2017 fibrous dysplasia can present in one bone monostotic or multiple bones polyostotic and can be associated with other conditions the lesions of fibrous dysplasia develop during skeletal formation and growth and have a variable natural evolution. Apr 01, 2009 fibrous dysplasia is a rare bone disorder presenting as a mononostotic or a polyostotic expanding fibrous skeletal lesion composed of boneforming mesenchyme. Transnasal endoscopic surgery for the treatment of fibrous 29 p.
Aug 04, 20 fibrous dysplasia may manifests as monostotic or polyostotic form. Fibrous dysplasia affects both cortical and cancellous bone and the bone marrow in a focal or diffuse manner. Maxillary lesions may extend to involve the maxillary sinus, zygoma, sphenoid bone, and floor of the orbit. The aim of this paper is to report a case of fibrous dysplasia of the maxillary sinus, describing its clinical presentation, radiological features. Maxillary osteosarcoma with fibrous dysplasia of frontal bone. Frontal bone was having the classical ground glass appearance suggestive of fibrous dysplasia of the bone. Panoramic radio introduction graphs showed a radiopaque expanded bone in the region of the posterior maxillary sinus and orbita. Cureus radiographic diagnosis of fibrous dysplasia in. Fibrous dysplasia fd, also known as lichtensteinjaffe disease, is a bone developmental anomaly due to a genetic defect in the osteoblastic differentiation caused by mutation in the gnas1 gene and characterized by a focal replacement of normal bone with fibrous stroma and. Most common site of occurrence with mandibular involvement is in the body portion. Case report the male sex, 11yearold, white patient from itaperuna, was forwarded to the otorhinolaryngology service of hsja with his responsible parent with a clinical picture of a progressive increase in the left hemiface. Best practice management guidelines for fibrous dysplasiamccune.
Pdf orthodontic treatment of patient with maxillofacial. The most common appearance of fibrous dysplasia on ct is an expanded bone showing a groundglass appearance fig. Craniofacial fibrous dysplasia cfd of the maxilla in an 11year old boy. Cone beam computed tomography scan revealed a mixed radiopacity that extended from the alveolar crest of the right posterior teeth to the right orbital. Fibrous dysplasia fd is a tumorlike growth that consists of replacement of the medullary bone with fibrous tissue, causing the expansion and weakening of the areas of bone involved. Enter your email address to receive your free pdf download. The patient had rotated maxillary second premolars. Scribd is the worlds largest social reading and publishing site. Fibrous dysplasia is a relatively rare tumorous lesion in the maxillofacial region. Fibrous dysplasia has been identified for more than a century, however the scientific and clinical interest for this pathology has been relatively low, probably because of its reduced incidence. The fibrous dysplasia is a benign bone disease, of slow growth and unknown etiology. It is more common for fibrous dysplasia to develop in. Materials and methods the authors bring their experience of.
An unusual case of fibrous dysplasia of the maxillary sinus. Pdf clinical guidelines for the management of craniofacial. Multidisciplinary approach for management of a patient. Fibrous dysplasia involving the skull base and temporal bone. Fibrous dysplasia an overview sciencedirect topics. Polyostotic fibrous dysplasia, which overall accounts for 20% of cases. Clinically, 11 of our 39 cases developed so rapidly that the lesions were suspected of being malignant. International journal of pediatric otorhinolaryngology, vol.
Diagnosis of craniofacial fibrous dysplasia should be considered in case of. The frontal process of the maxilla occasionally shows a localized fdlike appearance. Fibrous dysplasia fd is a rare bony disorder affecting one bone or multiple bones, and the affected bones are generally conceived to be less resistant to infection. Akintoye reported that 27 84% of 32 patients had craniofacial fd involving the jaws, and 31% had disease in both the maxilla and mandible. Fibrous dysplasia with cystic appearance in maxillary. Here we present a 16yearold female with a fibrous dysplasia in the maxilla. Diagnosis of polyostotic form is easier due to extraskeletal involvement. Fibrous dysplasia is usually a slowly progressive, benign disease that develops over several years and presents with deformity or mild symptomatology. Oral rehabilitation with dental implants for teeth involved. Radiographic diagnosis of fibrous dysplasia in maxilla cureus. Fibrous dysplasia is a rare bone disorder characterized by the replacement of normal bone by abnormal fibrous tissue. Five of 34 patients ages 421 years, who were subsequently diagnosed histologically as having fibrous dysplasia of the maxillary sinus, rapidly developed soft tissue masses of the malar region over a period of less than 4 months with.
As maxillary region swelling was more suggestive of sarcoma, it was taken as prime importance and treatment was planned. This page from great ormond street hospital gosh explains the causes, symptoms and treatment of fibrous dysplasia. Fibrous dysplasia fd is a condition in which normal bone marrow is replaced by an. Without visible scars and virtually free of postoperative functional disability, this approach offers good exposure of the middle third of the face for treatment of maxillary fibrous dysplasia with excellent cosmetic outcomes. The involvement of the craniofacial skeleton is not uncommon and, generally, produces facial asymmetries. Surgical resection for advanced bisphosphonaterelated. Craniofacial fibrous dysplasia the journal of laryngology. Notably, fd of the jaw bone with dental infections may be complicated by osteomyelitis 3, 4. Without visible scars and virtually free of postoperative functional. Jul 01, 2014 monofocal maxillary fibrous dysplasia with orbital, nasal and oral obstruction j plast reconstr aesthet surg, 63 2010, pp. Oct 19, 2017 the best sleeping position for back pain, neck pain, and sciatica tips from a physical therapist duration. Pdf fibrous dysplasia fd is an idiopathic skeletal disorder in which the.
Radiographic appearance of fibrous dysplasia associated with. Imaging findings of fibrous dysplasia with histopathologic. Midfacial degloving approach for management of the. Objective fibrous dysplasia fd is a benign bone disorder in facial bones. An unusual case of fibrous dysplasia of the maxillary. Fibrous dysplasia fd is a rare but important benign lesion affecting the maxillofacial region that presents bone pain, deformities, and fracture. Radiographically mixed radiolucentradiopaque lesions are seen in facial bones, frequently concerning the maxillary, mandible, sphenoid. Fibrous dysplasia is a tumor like developmental disorder with minimal chances of malignancies.
B, computed tomography scan demonstrating the extent of the maxillary defect. Fibrous dysplasia is a developmental anomaly that can affect any bone in the body. A 30yearold woman presented for orthodontic treatment, with a chief symptom of a shifting bite and concurrent facial asymmetry with aesthetic concerns. The gross appearance of fibrous dysplasia is a firm solid white mass replacing the medullary cavity. Maxillary osteosarcoma with fibrous dysplasia of frontal bone a rare case blog. Fibrous dysplasia of maxilla sumers radiology blog. Transnasal endoscopic surgery for the treatment of fibrous dysplasia of maxillary sinus associated to aneurysmal bone cyst in a 5yearold child. Pdf fibrous dysplasia fd is a nonmalignant condition caused by. Surgical removal of fibrous dysplasia lesions is generally reserved for equivocal cases or those with complications. Computed fibrous dysplasia fd is the abnormal development of tomography with threedimensional reconstruction dem bony tissue that leads to fibrous tissue proliferation. The skull and facial bones are the affected sites in 1025% of patients with monostotic fibrous dysplasia and in 50% of patients with polyostotic fibrous dysplasia. Most fibrous dysplasia cases were not obvious boundary but ground glass type. Fibrous dysplasia is a benign skeletal disorder in which the normal bone and marrow are replaced by fibrous tissue and haphazardly distributed woven bone. Cureus radiographic diagnosis of fibrous dysplasia in maxilla.
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