In this study, we examined radiographic patterns which. 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. May 24, 2012 fibrous dysplasia fd is a nonmalignant condition in which normal bone and marrow are replaced by fibrous tissue and haphazardly distributed woven bone 1, 2. The phenotype is variable and may be isolated to a single skeletal site or multiple sites. Surgery is most appropriate in cases where fractures are likely to occur, or where bones have become misshapen. Fibrous dysplasia is an uncommon bone disorder in which scarlike fibrous tissue develops in place of normal bone. The specific cause of fibrous dysplasia is unknown, but indications are that the disease is of developmental or congenital origin.
Monostotic fibrous dysplasia, though less serious than polyostotic fibrous dysplasia, is of greater concern to dentist because of the frequency in which the jaws are affected. The majority about 70% of people with fd have only one bone site involved, a condition called monostotic fd. This irregular tissue can weaken the affected bone and cause it to deform or fracture. Chronic untreated fibrous dysplasia of the mandible in a 40yearold man is described, with emphasis on the radiographic. Fibrous dysplasia of the left ethmoid sinus producing widening of the nasal bridge and proptosis of the left eye. The true incidence and prevalence of fibrous dysplasia are difficult to estimate, but the lesions are not rare. Base of the skull is the most common site of involvement in craniofacial fibrous dysplasia where sphenoid bone is most commonly affected.
The gross appearance of fibrous dysplasia is a firm solid white mass replacing the medullary cavity. Sep 17, 2014 fibrous dysplasia of the left ethmoid sinus producing widening of the nasal bridge and proptosis of the left eye. Fractures often require surgery, but can sometimes be treated with casting or splints. Maxilla is more commonly involved than fibrous dysplasia is of 3 types. This risk of fractures or bone deformity is higher in the long bones eg, femur, tibia, and humerus, but all the bones can be affected. The distinct kinds of fibrous dysplasia are monostotic fibrous dysplasia. Disease occurs along a broad clinical spectrum ranging from asymptomatic, incidental. Clinical and radiographic features of chronic monostotic fibrous. Jan 24, 2014 fibrous dysplasia conforms to the shape of the involved bone. Craniofacial fibrous dysplasia in an elderly patient. Fibrous dysplasia of the jaws wiley online library. It is a benign fibroosseous disease process in which normal bone is replaced by fibrous tissue and immature bone.
Fibrous dysplasia fd is a condition in which normal bone marrow is replaced by an abnormal proliferation of new fibrous connective tissue. 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 nord national organization for rare. Aug 04, 20 fibrous dysplasia usually get stabilized after puberty 1. In most cases, fibrous dysplasia occurs at a single site in one bone, but can occur at multiple sites in multiple bones. As a result, most complications result from fracture, deformity, functional impairment, and pain.
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. Typical microscopic findings include irregular spindles of woven bone, usually nonmineralized, scattered throughout a fibrocellular matrix. Abnormalities may involve a single bone monostotic form. Fibrous dysplasia is a skeletal disorder that is characterized by the replacement of normal bone with fibrous bone tissue. Fibrous dysplasia definition of fibrous dysplasia by. Fibrous dysplasia ossifying fibroma of the maxilla and. Swelling of the left maxilla extending from to maxillary tuberosity. The radiographic appearance of this lesion varies widely in the jaw. Axial ct scan showing the ground glass appearance of the fibrous dysplasia. Fibrous dysplasia fd is a rare bony disorder in which normal bone is replaced by abnormal fibroosseous tissue. Polyostotic fibrous dysplasia fibrous dysplasia fd of bone is a rare disease often associated with severe clinical outcome, including bone pain, deformities, and fractures. Introduction fibrous dysplasia fd is an uncommon developmental bone disease of benign origin. 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.
Patients may exhibit involvement of one bone monostotic fd. Radiographic appearance of fibrous dysplasia associated with. 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 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 dysplasia, maxilla, monostotic form, ground glass appearance. Bone affected by this disorder is replaced by abnormal scarlike fibrous connective tissue. The fibrous dysplasia is a benign bone disease, of slow growth and unknown etiology. Medications known as bisphosphonates are also used to. Clinical and radiographic features of chronic monostotic.
Fibrous dysplasia involving anterior maxilla with extension into antrum and wall, can present a leonine appearance. Fibrous dysplasia involves the maxilla almost twice as often as the mandible, frequenting the posterior region and is usually unilateral in nature. The involvement of the craniofacial skeleton is not uncommon and, generally, produces facial asymmetries. 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. Fibrous dysplasia article about fibrous dysplasia by the. Margins of the abnormal bone merge imperceptibly with the. Fibrous dysplasia fd is a fibroosseous lesion with no apparent familial, hereditary or congenital basis. Case report on a 14year quiescent phase260 examined in followup for the last 14 years.
Computed tomographic features of fibrous dysplasia of. The mucosa overlying the lesion seemed to be intact fig. Fibrous dysplasia is a relatively rare tumorous lesion in the maxillofacial region. 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 a term first suggested by lichtenstein and jaffe in 1942 of bone is a nonheritable disease in which abnormal tissue develops in place of normal bone. On clinical examination, the left maxilla was enlarged and the bone was hard on palpation. Disease occurs along a broad clinical spectrum ranging from asymptomatic, incidental lesions, to severe disabling disease. 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. The purpose of this study was to report six cases of fibrous dysplasia associated with the maxillary sinus.
The diagnosis of fibrous dysplasia is often made in infancy and childhood. Increased levels of serum alkaline phosphatase are observed in 40% of cases of polyostotic fibrous dysplasia and, more rarely, in monostotic form. Fibrous dysplasia fd is a sporadic benign skeletal disorder that can affect one bone monostotic. Pfd or they may have mccunealbright syndrome mas, which has been classically defined by the triad of pfd, cafeaulait skin macules. The most common sites of involvement include the rib, femur, tibia, mandible, skull, and humerus. Fibrous dysplasia is a benign intramedullary fibroosseous lesion originally described by lichtenstein1 in 1938 and by lichtenstein and jaffe in 19422. Imaging findings of fibrous dysplasia with histopathologic. Clinically, the monostotic form is characterized by a lytic expanding bone lesion, which may cause pain, deformity, fractures, and nerve entrapment. Fibrous dysplasia can be accompanied by endocrinopathies. Fibrous dysplasia of the facial bones and mandible. Recurrent monostotic fibrous dysplasia in the mandible. Jun 30, 2017 fibrous dysplasia is a skeletal disorder that is characterized by the replacement of normal bone with fibrous bone tissue. Fibrous dysplasia has been regarded as a developmental skeletal disorder characterized by replacement of normal bone with benign cellular fibrous connective tissue. Ground glass attenuation lesion filing right maxillary sinus and causing expansion of maxilla with extension to alveolar processes.
As a consequence of this bone fragility, patients have an increased 50% risk of fracture. It often involves the long bones, craniofacial bones, ribs, and pelvis. Gnas mutational analysis in differentiating fibrous. Fibrous displasia is a skeletal developmental disorder of the boneforming mesenchyme that manifests as a defect in osteoblastic differentiation and maturation. Fibrous dysplasia is an uncommon but important lesion affecting the maxillofacial region because it can cause severe deformity and asymmetry of face and jaw bones. Fibrous dysplasia has a varied radiographic appearance. May 31, 2016 fibrous dysplasia fd is a condition in which normal bone marrow is replaced by an abnormal proliferation of new fibrous connective tissue. Bone neoplasm, developmental, fibrous dysplasia, maxilla jaw are affected more. Generally, the occurrence rate is higher in the maxilla than in the mandible. It may involve one bone monostotic or multiple bones polyostotic. Fibrous dysplasia is a nonneoplastic developmental disease of bone, which is manifested as a defect in osteoblastic differentiation and maturation. Clinical and radiographic features of fibrous dysplasia of the mandible cysts occasionally occur in mature lesions of. Fibrous dysplasia is a disturbance of bone metabolism.
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. Fibrous dysplasia, maxilla, monostotic, polyostotic. 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. In mcdonaldjankowskis study, the most common radiographic presentation of. Five cases of fibrous dysplasia isolated to the facial bones and mandible are presented.
The treatment can be either conservative or complete resection. The monostotic form of fibrous dysplasia comprises approximately 80% of all cases and is seen in patients between 10 and 70 years old. Jul 29, 2019 fibrous dysplasia is an uncommon bone disorder in which scarlike fibrous tissue develops in place of normal bone. Clinical guidelines for the management of craniofacial. Radiographic appearance of fibrous dysplasia associated. Jun 30, 2017 unfortunately, there is no cure for fibrous dysplasia. Fibrous dysplasia conforms to the shape of the involved bone. Orthopedic surgery for fibrous dysplasia clinical presentation. Fibrous dysplasia involving the right maxillary sinus and turbinate. It is a nonneoplastic developmental hamartomatous disease of the bone, characterised by a blend of fibrous and osseous elements in the region. Here a case of fibrous dysplasia of maxillary bone has reported. Fibroosseous lesions of bonesfibrous dysplasia authorstream. Fibrous dysplasia of the facial bones and mandible springerlink.
Fibrous dysplasia is a benign noncancerous bone condition in which abnormal fibrous tissue develops in place of normal bone. Orthodontic treatment of patient with maxillofacial fibrous dysplasia. The affected bone becomes enlarged, brittle and warped. Orbital scoringtransposition supraorbital fibrous dysplasia dr. Fibrous dysplasia is a rare bone disease that represents 2. The etiology of fibrous dysplasia has been linked to an activating mutation in the gene that encodes the. Fibrous dysplasia fd is a nonmalignant condition caused by postzygotic, activating mutations of the gnas gene that results in inhibition of the differentiation and proliferation of boneforming stromal cells and leads to the replacement of normal bone and marrow by fibrous tissue and woven bone. The ct scan of this patient and his surgery are depicted below. Pdf fibrous dysplasia fd is a bone development anomaly characterized by hamartomatous.
Fibrous displasia is a skeletal developmental disorder of the boneforming mesenchyme that manifests as a. 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. Oct 19, 2017 orbital scoringtransposition supraorbital fibrous dysplasia dr. What is fibrous dysplasia childrens hospital colorado. Surgical removal of fibrous dysplasia lesions is generally reserved for equivocal cases or those with complications. Fibrous dysplasia fd is an uncommon developmental bone disease of benign origin.
Fibrous dysplasia is a condition that causes abnormal growth or swelling of bone. Monostotic fibrous dysplasia of the maxilla semantic scholar. As children grow, affected bone may become misshapen dysplastic. It causes bone pain, deformities, and pathological fractures. It may involve one bone or multiple bones polyostotic. 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. Pictures of fibrous dysplasia of the sinuses otolaryngology.
In general they affect 1 in 4,000 to 10,000 individuals 3. Fibrous dysplasia usually get stabilized after puberty 1. Approximately 30% of monostotic fd mfd lesions are found in the cranial or facial bones. Maxillary frenectomy and free gingival graft in mandibular anterior region were performed by a periodontist. Female patient, white, 20 years old, attended the dental clinic reporting a slow increase in volume in the right mandible region over the last 5 years. Fibrous dysplasia may involve one bone monostotic form or, less commonly, multiple bones polyostotic form. Fibrous dysplasia lesions are characterized by woven ossified tissue and extensive marrow fibrosis. Fibrous dysplasia is a condition characterized by abnormal bone growth. Fibrous dysplasia is a non inherited skeletal disorder in which boneforming cells fail to mature and produce too much fibrous or connective tissue.
Fibrous dysplasia and ossifying fibroma of the jaws are fibroosseous lesions with different clinical course and treatment strategies. Fibrous dysplasia genetic and rare diseases information. Clinical guidelines for the management of craniofacial fibrous. Monostotic type of fibrous dysplasia of maxilla suresh kumar dhonvan1, pavan kumar b2, srinivas gadipelly3, haripriya chari4 abstract. Special forms of fibrous dysplasia leontiasis ossea. This abnormal fibrous tissue weakens the bone, making it abnormally fragile and prone to fracture. Fibrous dysplasia is a rare bone disorder presenting as a mononostotic or a polyostotic expanding fibrous skeletal lesion composed of boneforming mesenchyme. Fibrous dysplasia can occur in any part of the skeleton but the bones of the skull and face, thigh, shin, ribs, upper arm and pelvis are most commonly affected. The skull is frequently affected as are legs, arms, and ribs.1567 694 722 482 1333 171 624 1109 333 827 134 641 161 1026 28 551 949 142 1480 1441 871 844 104 1272 751 1033 232 683 1410 846 1057 1172 1459 99 36 1049