Fibrous dysplasia is a rare bone disease that represents 2. Fibrous dysplasia of the maxilla and mandible sciencedirect. This study evaluates the possibility of diagnosing fibrous dysplasia on imaging alone, without biopsy of the lesion, which is often burdensome for the patient. Monostotic type of fibrous dysplasia of maxilla suresh kumar dhonvan1, pavan kumar b2, srinivas gadipelly3, haripriya chari4 abstract. Fibrous dysplasia and ossifying fibroma of the paranasal sinuses volume 112 issue 10 d. Pdf fibrous dysplasia fd is a bone development anomaly characterized by hamartomatous proliferation of fibrous tissue.
Due to somatic mutation of cfos oncogene that causes activation of camp pathway. It may involve one bone monostotic or multiple bones polyostotic. It can be monostotic or polyostotic, affecting maxilla twice as compared to mandible. In summary, fibrous dysplasia involving the skull base can present in myriad ways. Craniofacial fibrous dysplasia radiology reference article. It represents with three forms namely monostotic single bone involvement, polyostotic multiple 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. Fibrous dysplasia is a skeletal disorder in which boneforming cells fail to mature and produce too much fibrous, or connective, tissue. On clinical examination, the left maxilla was enlarged and the bone was hard on palpation. Fibrous dysplasia of the upper jaw and cheekbone maxilla and zygoma surgery. Fibrous dysplasia results when fibrous tissue develops in a section of bone, causing it to become deformed. Fibrous dysplasia and cherubism pubmed central pmc.
Fibrous dysplasia may be present in monostotic or polyostotic form, or albrights syndrome,57,1517. The common sites affected by the monostotic form in craniofacial bones are frontal, sphenoid, ethmoid, maxilla and mandible bones 10,17. Jun 06, 2016 introduction the use of digital planning in the practice of oral and maxillofacial surgery has afforded clinicians with enhanced treatment planning capabilities through the fabrication of surgical stents, which results in reduced intraoperative time and improved predictability of surgical outcomes. Fibrous dysplasia science and education publishing. As a result, most complications result from fracture, deformity, functional impairment, and pain. Modern imaging modalities and histopathologic analysis have made diagnosis relatively straightforward.
The frontal process of the maxilla occasionally shows a localized fdlike appearance. Fibrous dysplasia fd is a rare bony disorder in which normal bone is replaced by abnormal fibroosseous tissue. Craniofacial fibrous dysplasia is different in that it ignores suture lines. Fibrous dysplasia of the skull base fibrous dysplasia of the frontal and sphenoid bones eventually leads to distortion of facial features and skull shape as a result of the proliferation of thick dense bone. The purpose of this study was to determine its prevalence and findings on ct. Fibrous dysplasia overview nih osteoporosis and related.
Fibrous dysplasia of the facial skeleton commonly involves the maxilla. Fibrous dysplasia is a congenital present at birth condition that affects bone growth and development. 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. Fibrous dysplasia is a nonneoplastic hamartomatous developmental fibroosseous lesion of bone. In most cases, fibrous dysplasia occurs at a single site in one bone, but can occur at multiple sites in multiple bones. Fibrous dysplasia, maxilla, ground glass appearance.
Approximately 30% of monostotic fd mfd lesions are found in the cranial or facial bones. Fibrous dysplasia is a common benign skeletal lesion that may involve one bone monostotic or multiple bones polyostotic and occurs throughout the skeleton with a predilection for the long bones, ribs, and craniofacial bones. Fibrous dysplasia is first diagnosed in infancy and. Fibrous dysplasia of the upper jaw and cheekbone, maxilla. Fibrous dysplasia, maxilla, monostotic form, ground glass appearance. Fibrous dysplasialike appearance of the frontal process. The replacement of normal bone in fibrous dysplasia can lead to pain, misshapen bones, and fracture, especially when it occurs in the long bones. The aim of this paper is to report a case of fibrous dysplasia of the maxillary sinus, describing its clinical presentation, radiological features, histopathological appearance and surgical. The most common location is the middle part of the tibia shin, although the fibula a smaller bone in the calf and the long bones in the arm humerus, radius, or ulna may also be affected. Treatment of fibrous dysplasia utilizing digital planning. Aggressive fibrous dysplasia of the maxillary sinus. 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. It arises from postzygotic gainoffunction mutations in the gnas gene, which encodes the.
Fibrous dysplasia is a benign bone lesion characterized by replacement of normal bone by fibrous connective tissue, and its diagnosis is based on clinical, radiological and histological findings. Fibrous dysplasia is a skeletal disorder that is characterized by the replacement of normal bone with fibrous bone tissue. 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. 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. The purpose of this study was to report six cases of fibrous dysplasia associated with the maxillary sinus. Instead of maturing into solid bone, affected bones stay at the immature fibrous stage so are weak and misshapen. Extension into the maxillary antrum usually occurs from the lateral wall, and the last section of the sinus to be involved usually is. Eight of the specimens were circumscribed tumors removed in one piece. Fibrous dysplasia is characterized by excessive proliferation of boneforming mesenchymal cells.
Fibrous dysplasia of the mandible fibrous dysplasia report george w. Note the inferior alveolar malposition and protrusion along with the poor dental status and evidence of recent bleeding from the aveolus. Fibrous dysplasia is a rare bone disorder characterized by the replacement of normal bone by abnormal fibrous tissue. Surgery, particularly in a challenging region such as the skull base, should be reserved for patients with functional impairment or a cosmetic deformity.
Orbital scoringtransposition supraorbital fibrous dysplasia dr. Fibrous dysplasia fd is an idiopathic skeletal disorder in which the trabecular bone is replaced and distorted by poorly organized, structurally unsound fibroosseous tissue. Fibrous dysplasia is an uncommon bone disorder in which scarlike fibrous tissue develops in place of normal bone. Fibroosseous lesions of bonesfibrous dysplasia authorstream. Radiographic appearance of fibrous dysplasia associated with. The mucosa overlying the lesion seemed to be intact fig. 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. 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.
Fibrous dysplasia is a nonneoplastic developmental disease of bone, which is manifested as a defect in osteoblastic differentiation and maturation. 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. This is a 23yearold patient from mizoram, india who first noticed the swelling on the left side of his face about four months ago. Fibrous dysplasia is a nonneoplastic developmental disease of bone, which manifest as a defect in osteoblastic differentiation and maturation. Best practice management guidelines for fibrous dysplasia. Fibrous dysplasia and ossifying fibroma an advent in. Fibrous dysplasia of the maxilla or mandible may cause the displacement of permanent teeth, interfere with the eruption of new teeth, and contribute to malocclusion. Fibrous dysplasia may expand into the antrum by displacing its cortical boundary and subsequently occupying part or most of the maxillary sinus. Swelling of the left maxilla extending from to maxillary tuberosity. 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 is helpful in distinguishing fibrous dysplasia from a malignancy. Jun, 20 objective fibrous dysplasia fd is a benign bone disorder in facial bones.
In 1938, lichtenstein first coined the term fibrous dysplasia fd. 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. Development of a facial swelling in a patient from mizoram, india. It may involve one bone or multiple bones polyostotic. 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. Jan 24, 2014 pelvis fibrous dysplasia expansile, lytic, bubbly lesion.
Aug 04, 20 fibrous dysplasia usually get stabilized after puberty 1. 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. Surgery is the treatment of choice with two available options. This page from great ormond street hospital gosh explains the causes, symptoms and treatment of fibrous dysplasia.
Jun 30, 2017 fibrous dysplasia is a skeletal disorder that is characterized by the replacement of normal bone with fibrous bone tissue. It is a nonneoplastic developmental hamartomatous disease of the bone, characterised by a blend of fibrous and osseous elements in the region. Fibrous dysplasia genetic and rare diseases information. This is a rare condition that can be skillfully treated at the international craniofacial institute in dallas, texas, as these before and after photographs clearly show. Fibrous displasia is a skeletal developmental disorder of the boneforming mesenchyme that manifests as a. In this study, we examined radiographic patterns which. Radiographic diagnosis of fibrous dysplasia in maxilla. It is termed dysplasia because of the inability of the involved tissue to form mature lamellar bone from the immature woven precursor.
Maxillary osteosarcoma with fibrous dysplasia of frontal. Oct 19, 2017 orbital scoringtransposition supraorbital fibrous dysplasia dr. Early diagnosis and adequate surgical resection are keys for high survival rate. These are not well circumscribed and commonly extend locally to involve maxillary sinus, zygomatic process, orbital floor and skull base resulting in severe malocclusion and marked facial asymmetry. The aim of this study was to report a case of unilateral fibrous dysplasia in the maxilla, in the palate region, by using. Benign fibro osseous condition involving one or more bones of the cranial and extracranial skeleton, consists of nonencapsulated lesions which show replacement of normal bone by cellular fibrous tissues edwards 1984 originally introduced by lichtenstein in 1938 one of the most perplexing diseases of osseous tissues. Fibrous dysplasia of maxilla has marked predilection for children. 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.
Fibrous dysplasia of the maxilla, journal of pediatric. The involvement of the craniofacial skeleton is not uncommon and, generally, produces facial asymmetries. It has now become evident that fibrous dysplasia is a genetic disease caused by somatic activating mutation of the gs. 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. Fibrous dysplasia is usually a slowly progressive, benign disease that develops over several years and presents with deformity or mild symptomatology. The etiology of fibrous dysplasia has been linked to an activating mutation in the gene that encodes the. This paper documents a rare presentation in which there is osteosarcoma of the maxilla with fibrous dysplasia of frontal bone, dual presentation in the same patient. Fibrous dysplasia of the maxilla fibrous dysplasia of the maxilla godse, a. Rib fibrous dysplasia expansile lytic lesion with no rib destruction. 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. The fibrous dysplasia is a benign bone disease, of slow growth and unknown etiology.
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. Fibrous dysplasia fd is a fibroosseous lesion with no apparent familial, hereditary or congenital basis. Fibrous dysplasia has a varied radiographic appearance. 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. It often involves the long bones, craniofacial bones, ribs, and pelvis. Fibrous dysplasia fd is an uncommon developmental bone disease of benign origin. Multidisciplinary approach for management of a patient. Case report on a 14year quiescent phase260 examined in followup for the last 14 years. Here we present a 16yearold female with a fibrous dysplasia in the maxilla. Milford skip to main content accessibility help we use cookies to distinguish you from other users and to provide you with a better experience on our websites. Maxilla is more commonly involved than fibrous dysplasia is of 3 types. Fibrous displasia is a skeletal developmental disorder of the boneforming mesenchyme that manifests as a defect in osteoblastic differentiation and maturation. 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.
Areas of healthy bone are replaced with this fibrous tissue. This irregular tissue can weaken the affected bone and cause it to deform or fracture. The maxilla is the most commonly affected facial bone, with facial asymmetry being the usual complaint. Fibrous dysplasia fd is a benign, non neoplastic disease that develops in all parts of the body. Fibrous dysplasia has been regarded as a developmental skeletal disorder characterized by replacement of normal bone with benign cellular fibrous connective tissue. Management of aggressive midface and orbital fibrous dysplasia. Oct 20, 2016 osteofibrous dysplasia is a rare, noncancerous benign tumor that affects the long bones. Fibrous dysplasia and ossifying fibroma of the paranasal. The disease seems to stabilize after bone maturation3. In the monostotic form, craniofacial bones are involved in about 10% of the cases. The areas of fibrous tissue are interwoven with newly formed bone trabeculae that vary in size and shape.
A, preoperative view of patient with endstage right maxillary fibrous dysplasia. Ct scan shows a lesion that is confined to the bone with no soft tissue component. It is impossible to eradicate without a radical surgery, which is mutilating. The radiographic appearance of this lesion varies widely in the jaw.
Fibrous dysplasia involving the skull base and temporal bone. Introduction fibrous dysplasia fd is an uncommon developmental bone disease of benign origin. 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. At molecular level fd is caused by sporadic mutation of a subunit of the stimulatory g protein. Fibrous dysplasia of the mandible pdf free download.
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. Fibrous dysplasia is a relatively rare tumorous lesion in the maxillofacial region. However deeper evaluation of the disease and its treatment outcome study should be carried out on multicentirc basis and with longer follow up period keywords fibrous dysplasia, maxilla, monostotic form, ground glass form. Here a case of fibrous dysplasia of maxillary bone has reported.
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. Generally, the occurrence rate is higher in the maxilla than in the mandible. In most cases, bone dysplasia is diagnosed incidentally as a result of facial asymmetry, facial. Osteosarcomas of jaw differ from osteosarcomas of other long bones in its biological behaviour. Fibrous dysplasia fd is a sporadic benign skeletal disorder that can affect one bone monostotic form or multiple bones polyostotic form. Fd is a rare, nonmalignant condition in which normal bone and marrow are replaced by fibrous tissue and randomly distributed woven bone, usually with pain, bony deformity, and pathologic fractures.