This was a study of 38 patients with fibrous dysplasia of the lesser wing of the sphenoid bone. In the jaws, fibrous dysplasia is more frequent in the maxilla than in the mandible and can cause severe deformities and blindness7. Fibrous dysplasia fd is a benign bone lesion characterized by. Fibrous dysplasia fd is a pathologic condition in which normal bone is altered by abnormal fibroosseous tissue, causing distortion and overgrowth of the affected bone. The most prominent condition in the differential diagnosis is fibrous dysplasia. It often involves the long bones, craniofacial bones, ribs, and pelvis. Fibrous dysplasia fd is an uncommon skeletal disorder in which normal bone is replaced by abnormal fibroosseous tissue. Fibrous dysplasia of bone frequently involves the anterior base of the cranium and results in encasement of the opticnerve canals. Fibrous dysplasia is a benign fibroosseous lesion of obscure pathogenesis characterized by formation of fibrous connective tissue within the spongiosa of the affected bone and. Craniofacial fibrous dysplasia is one of four types of fibrous dysplasia and is characterized, as the name suggests, by involvement of the skull and facial bones for a general discussion of the underlying pathology, refer to the parent article fibrous dysplasia. Aug 04, 20 fibrous dysplasia usually get stabilized after puberty 1. The lesion is poorly circumscribed and initially radiolucent coleman et al.
Fibrous dysplasia is a skeletal disorder that is characterized by the replacement of normal bone with fibrous bone tissue. The primary tool for diagnosis of fibrous dysplasia is an xray. Fibrous dysplasia of the maxilla fibrous dysplasia of the maxilla godse, a. We describe one case of maxillary bone fibrous dysplasia that, in spite of its benign nature, caused facial deformity. Fibrous dysplasia fd is a benign fibroosseous disease which causes replacement of normal bone by fibrous connective tissue and immature bone, usually affecting the children and young adults. Here we present a 16yearold female with a fibrous dysplasia in the maxilla and obliteration of the sinus in the same side. The monostotic form of fibrous dysplasia comprises approximately 80% of all cases and is seen in patients between 10 and 70 years old. Fibrous dysplasia fd is a nonneoplastic tumorlike congenital process, manifested as a localised defect in osteoblastic differentiation and maturation, with the replacement of normal bone with large fibrous stroma and islands of immature woven bone. Fibrous dysplasia versus juvenile ossifying fibroma.
Bone affected by this disorder is replaced by abnormal scarlike fibrous connective tissue. It is a benign condition characterized by replacement of bone by disorganized fibrous tissue and immature bone which. Progressive fibrous dysplasia of the maxilla harold e. Fibrous dysplasia of the maxilla and mandible sciencedirect. This abnormal fibrous tissue weakens the bone, making it abnormally fragile and prone to fracture. Fibrous dysplasia has a varied radiographic appearance. Request pdf fibrous dysplasia of the maxilla fibrous dysplasia is a rare disorder of the bone. The involvement of the craniofacial skeleton is not uncommon and, generally, produces facial asymmetries. Fibrous dysplasia fd is an uncommon developmental bone disease of benign origin. Approximately 30% of monostotic fd mfd lesions are found in the cranial or facial bones. Fibrous dysplasia overview nih osteoporosis and related. Fibrous dysplasia is first diagnosed in infancy and childhood 7, mainly in the first and second decades 8. It is seen in 2 main forms of presentation, monoostotic and the polyostotic form with cafe au lait spots and precocious puberty mccunealbright syndrome.
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. Fibrous dysplasia is a benign intramedullary fibroosseous lesion originally described by lichtenstein1 in 1938 and by lichtenstein and jaffe in 19422. Objective fibrous dysplasia fd is a benign bone disorder in facial bones. Fibrous dysplasia is a disorder where normal bone and marrow is replaced with fibrous tissue, resulting in formation of bone that is weak and prone to expansion. The areas of fibrous tissue are interwoven with newly formed bone trabeculae that vary in size and shape. The child was later referred to the maxillofacial unit after about 2 months of unsuccessful management by the pediatricians. The fibrous dysplasia is a benign bone disease, of slow growth and unknown etiology. In polyostotic form, patients usually present by 10 years old. The condition may be diagnosed, therefore, even in a person with no symptoms who is getting an xray for other reasons. It may involve one bone monostotic or multiple bones polyostotic. Fibrous dysplasia is a developmental tumor like condition in which normal bone is replaced by excessive proliferation of cellular fibrous connective tissue intermixed with irregular bony trabeculae. Fibrous dysplasia fd is a rare bony disorder in which normal bone is replaced by abnormal fibroosseous tissue.
Fd involves the maxilla almost two times more often than the mandible. Fibrous dysplasia of the maxilla, journal of pediatric. Fibrous dysplasia fd is a benign bone lesion characterized by the progressive replacement of. It is seen in 2 main forms of presentation, monoostotic and the polyostotic form with cafe au. Library of published research fibrous dysplasia foundation. Case report on a 14year quiescent phase260 examined in followup for the last 14 years. Midfacial degloving approach for management of the. As children grow, affected bone may become misshapen dysplastic. Fibrous dysplasia is a nonneoplastic hamartomatous developmental fibroosseous lesion of bone.
Fibrous dysplasia is a mosaic disease resulting from postzygotic activating mutations of the gnas locus at 20q. The fibrous dysplasia fd is defined as a benign osseous disease characterized by a process of normal bone reabsorption, followed by an abnormal proliferation of a disorganized fibroosseous tissue 1. Fibrous displasia is a skeletal developmental disorder of the boneforming mesenchyme that manifests as a defect in osteoblastic differentiation and maturation. Ashok department of oral medicine and radiology, bapuji dental college and hospital, davangere, karnataka, india corresponding author abstract introduction bone is a dense calcified tissue, which is specifically affected by a variety of diseases. Report of two cases nisha dua 1, neetu singla 2, saloni arora 2, shaveta garg 2 1 department of oral medicine and radiology, sri sukhmani dental college and hospital, dera bassi, punjab, india 2 department of oral medicine and radiology, guru nanak dev dental college and research institute, sunam, punjab, india.
This study evaluates the possibility of diagnosing fibrous dysplasia. Craniofacial fibrous dysplasia radiology reference article. The most common sites of involvement include the rib, femur, tibia, mandible, skull, and humerus. Midfacial degloving approach for management of the maxillary fibrous dysplasia. Fibrous dysplasia is a benign bone lesion characterized by replacement of normal bone by fibrous connective tissue, and its. Fibrous dysplasia usually get stabilized after puberty 1. Fibrous dysplasia nord national organization for rare. In this lesion, abnormal growth or swelling of the bone is a signi.
Apr 01, 2009 fibrous dysplasia of the maxilla fibrous dysplasia of the maxilla godse, a. Fibrous dysplasia is a rare bone disorder characterized by the replacement of normal bone by abnormal fibrous tissue. Here, we are presenting two case reports of fd involving the maxilla. Fibrous dysplasia is a congenital present at birth condition that affects bone growth and development. The diagnosis of fibrous dysplasia and ossifying fibroma is made on a combination of clinical, radiological and pathological criteria. Fibrous dysplasia of the maxilla in an elderly female. 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. It causes bone pain, deformities, and pathological fractures. As a result, most complications result from fracture, deformity, functional impairment, and pain. 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 genetic and rare diseases information. The true incidence and prevalence of fibrous dysplasia are difficult to estimate, but the lesions are not rare.
We report a case of polyostotic fibrous dysplasia in a male patient. Pdf fibrous dysplasia of maxilla ijoart editor academia. Fibrous dysplasia is first diagnosed in infancy and. Fibrous dysplasia, maxilla, monostotic, polyostotic. On clinical examination, the left maxilla was enlarged and the bone was hard on palpation. Jul 29, 2019 fibrous dysplasia is an uncommon bone disorder in which scarlike fibrous tissue develops in place of normal bone. Fibrous dysplasia fd is a nonneoplastic tumorlike congenital process, manifested as a localized defect in osteoblastic differentiation and maturation, with the replacement of normal bone with large fibrous stroma and islands of immature woven bone.
Diagnosis the most common presenting symptom in fibrous dysplasia is a gradual, painless enlargement of the involved bone or bones in the craniofacial region, clinically seen as facial asymmetry. Cureus radiographic diagnosis of fibrous dysplasia in maxilla. Fibrous dysplasia involving the right maxillary sinus and turbinate. Areas of healthy bone are replaced with this fibrous tissue. Fibrous dysplasia involves the maxilla almost twice as often as the mandible, frequenting the posterior region and is usually unilateral in nature. Fibrous dysplasialike appearance of the frontal process of. Fibrous dysplasia belongs to a group of fibroosseous lesions in which the normal bone is replaced by cellular fibrous connective tissue stroma. Fibrous dysplasia and ossifying fibroma an advent in their. Disease occurs along a broad clinical spectrum ranging from asymptomatic, incidental lesions, to severe disabling disease. Fibrous dysplasia is a nonneoplastic developmental disease of bone, which is manifested as a defect in osteoblastic differentiation and maturation.
Imaging findings of fibrous dysplasia with histopathologic. Here a case of fibrous dysplasia of maxillary bone has reported. While bone appears solid in an xray, a fibrous dysplasia lesion has a relative distinct appearance often described as ground glass. Fibrous dysplasia of the maxilla jama otolaryngology. Monostotic fibrous dysplasia is more common than the polyostotic form and usually involves jaw bones, ribs and femur. Ramsey, md doctor ramsey is chief of tumor surgery, usphs hospital, baltimore, 444 e68th st, new york doctor ramsey is chief of tumor surgery, usphs hospital, baltimore 444 e68th st new york requests for reprints should be directed to doctor ramsey at usphs hospital, 3100 wyman park dr, baltimore, 21211.
This irregular tissue can weaken the affected bone and cause it to deform or fracture. Radiographic diagnosis of fibrous dysplasia in maxilla. In general they affect 1 in 4,000 to 10,000 individuals 3. Progressive fibrous dysplasia of the maxilla sciencedirect. It is a benign condition characterized by replacement of bone by disorganized fibrous tissue and immature bone which, histologically, has numerous degrees of metaplasia. Fibrous dysplasia is a nonneoplastic developmental disease of bone, which manifest as a defect in osteoblastic differentiation and maturation.
Fibrous dysplasia fd is a sporadic benign skeletal disorder that can affect one bone monostotic. It can involve a single site monostotic fibrous dysplasia, multiple sites polyostotic fibrous dysplasia5 or occur as part of the mccune albright6. Fibrous dysplasia is characterized by excessive proliferation of boneforming mesenchymal cells. It frequently appears in the posterior region of the jaw bone and is usually unilateral. Fibrous dysplasia, maxilla, radiographic feature 1. Fibrous dysplasia and ossifying fibroma of the paranasal. The frontal process of the maxilla occasionally shows a localized fdlike appearance. It is a sporadic condition it is a sporadic condition that results from a postzygotic mutation in the gnas1 2 gene protein linked to the guanine nucleotide. Monostotic type of fibrous dysplasia of maxilla suresh kumar dhonvan1, pavan kumar b2, srinivas gadipelly3, haripriya chari4 abstract. Fibrous dysplasialike appearance of the frontal process. Fibrous dysplasia affecting maxilla in a year old. Fibrous dysplasia is a benign fibroosseous lesion characterised by the replacement of normal bone by excessive proliferation of cellular fibrous connective tissue which is slowly replaced by bone, osteoid, or cementumlike material. Fibrous displasia is a skeletal developmental disorder of the boneforming mesenchyme that manifests as a.
A dilemma the incisional biopsy was jubenile from the lesion and the pathology process was reported as jof. In most cases, fibrous dysplasia occurs at a single site in one bone, but can occur at multiple sites in multiple bones. Fibrous dysplasia has 4 different disease patterns monostotic 70%. The most common sites are the bones in the skull and face, the long bones in the arms and legs, the pelvis, and the ribs. The disease seems to stabilize after bone maturation3. It may involve one bone or multiple bones polyostotic. Fibrous dysplasialike appearance of the frontal process of the maxilla on ct. Fibrous dysplasia and ossifying fibroma an advent in. Cbct features and histopathological examination of fibrous. Polyostotic fibrous dysplasia, cafe au lait skin pigmentation large, dark lesions with serpiginous coast of maine borders in chest, neck, back, almost exclusively in women also endocrine abnormalities precocious puberty, hyperthyroidism, pituitary adenomas that secrete growth hormone primary adrenal hyperplasia. It can be monostotic or polyostotic, affecting maxilla twice as compared to mandible. Introduction fibrous dysplasia fd is an uncommon developmental bone disease of benign origin. Fibrous dysplasia of bone wikipedia in the absence of clinical signs, reevaluation with panoramic radiographs every 2 or 3 years is adequate beylouni et al.
This page from great ormond street hospital gosh explains the causes, symptoms and treatment of fibrous dysplasia. Sometimes, the classic clinical, radiologic or pathologic features of fibrous dysplasia or ossifying fibroma may not be evident, but overlapping features of both may be seen. The lesion is firstly described by lichtenstein 3 in 1938. Midfacial degloving approach for management of the maxillary.
Fibrkma of the fd lesions from juvenile ossifying fibroma is critical because the treatment protocols are entirely different in these two. Jun 30, 2017 fibrous dysplasia is a skeletal disorder that is characterized by the replacement of normal bone with fibrous bone tissue. The maxilla is the most commonly affected facial bone, with. Our case is a monostotic type of fibrous dysplasia involving left maxilla, managed by surgery in which the fibrous dysplas tic bone was removed by osteotomy. It has been assumed that such encasement leads to constriction and eventual blindness. Cone beam computed tomography findings in fibrous dysplasia. It represents with three forms namely monostotic single bone involvement, polyostotic multiple bone. 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. Fibrous dysplasia fd is a benign fibroosseous pathologic condition characterized by the replacement of bone with fibrous tissue 1,2. Fibrous dysplasia is a skeletal disorder in which boneforming cells fail to mature and produce too much fibrous, or connective, tissue.
The lesion may involve one or more bones being the maxilla the facial bone more affected. Fibrous dysplasia is an uncommon bone disorder in which scarlike fibrous tissue develops in place of normal bone. Fibrous dysplasia is a bone disorder of unknown origin characterized by slow. Clinical guidelines for the management of craniofacial fibrous. Fibrous dysplasia, maxilla, monostotic form, ground glass appearance. Fibrous dysplasia diagnosis and treatment mayo clinic. The purpose of this study was to determine its prevalence and findings on ct.
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