Fibrous dysplasia fd is a rare bony disorder in which normal bone is replaced by abnormal fibroosseous tissue. Malignant transformation of fibrous dysplasia of the maxilla malignant transformation of fibrous dysplasia of the maxilla varghese, a. Congenital malformations of nose and paranasal sinuses. The dental phenotype includes problems in both tooth formation multiple supernumerary permanent teeth and tooth eruption lack of shedding of primary teeth and delayed or arrested eruption of permanent teeth. Fibrous dysplasia involving the skull base and temporal.
Mandible and maxilla can present a wide variety of pathology with diverse origin, including tumoral, infectious, traumatic, metabolic or congenital lesions. Aggressive fibrous dysplasia of the maxillary sinus. The frontonasal prominence gives rise to inferior mesodermic projection which goes on to form. The areas of fibrous tissue are interwoven with newly formed bone trabeculae that vary in size and shape. Cleidocranial dysplasia ccd, caused by mutations in the runt. Fibrous dysplasia is a nonneoplastic lesion of unknown origin with onefourth involving head and neck. 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. About about europe pmc funders joining europe pmc governance roadmap outreach.
Fibrous dysplasia, maxilla, ground glass appearance. It affects the bones of the cranium and face in 3 ways. In summary, fibrous dysplasia involving the skull base can present in myriad ways. Sphenoid dysplasia can be seen isolated or associated with an underlying plexiform neurofibroma pnf. Clinical guidelines for the management of craniofacial. 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 can occur at any age, but typically is noticed during mid to late childhood. 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. Polyostotic forms occur but in the facial area the monostotic type is the rule and represents 90 percent of all cases of fibrous dysplasia.
Kanazawa university 2 department of oral surgery, school of medicine, gyifu university. The fibrous dysplasia is a benign bone disease, of slow. Fibrous dysplasia is a skeletal disorder that is characterized by the replacement of normal bone with fibrous bone tissue. Children with cherubism vary in severity in their maxilla and mandible bony lesions. The premolars may be congenitally missing, and the primary teeth baby teeth may be. Florid cement osseous dysplasia and chronic diffuse. It appears in the otolaryngic area chiefly in the maxilla, but also in the mandible, ethmoids, frontal and temporal bones. There are many variations of a type of clefting and classifications are needed to describe and classify all types of clefting. Presence of crownradicular anomalies and multidisciplinary approach of a clinical case romeo u, galluccio g, palaia g, tenore g, carpenteri f, barbato e, polimeni a department of oral and maxillofacial sciences, sapienza university of rome, rome, italy.
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. Characteristic radiological and histological patterns of. Fibrous dysplasia fd is a nonneoplastic, expansile lesion of unknown origin. The lesion is radioopaque with some radiolucent mottling. Fibrous dysplasia and osteofibrous dysplasia are both benign fibro. The patient had rotated maxillary second premolars, moderate. 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. Here we present a 16yearold female with a fibrous dysplasia in the maxilla. 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. Florid cement osseous dysplasia and chronic diffuse osteomyelitis in maxilla. Approximately 30% of monostotic fd mfd lesions are found in the cranial or facial bones. Sphenoid bone dysplasia is a distinctive, although uncommon, manifestation of nf1.
Fibrous dysplasia, a benign, nonneoplastic disease, is seen in all parts of the body. The fibrous dysplasia is a benign bone disease, of slow growth and unknown etiology. In these cases, the expression of proliferating cell nuclear antigen pcna and the presence of argyrophilic nucleolar organizer regions agnor, as well as dna. Comparative study of fibrous dysplasia and osteofibrous.
Fibrous dysplasia of the maxilla fibrous dysplasia of the maxilla godse, a. To characterize the diagnostic features of ossifying fibroma of and fibrous dysplasia fd of the jaw bones. Fibrous dysplasia is usually a slowly progressive, benign disease that develops over several years and presents with deformity or mild symptomatology. Fibrous displasia is a skeletal developmental disorder of the boneforming mesenchyme that manifests as a. Malignant changes with fibrous dysplasia include osteosarcoma. The most common area of the craniofacial skeleton to be affected is the orbit. Fibrous dysplasia of the maxilla and mandible sciencedirect. Fibrous dysplasia is a benign intramedullary fibroosseous lesion originally described by lichtenstein1 in 1938 and by lichtenstein and jaffe in 19422. The true incidence and prevalence of fibrous dysplasia are difficult to estimate, but the lesions are not rare. The cause is unknown, and the disease affects the jaws, teeth, and adjacent soft tissue.
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. Management of aggressive midface and orbital fibrous dysplasia. In about onefourth of cases this disorder affects the head and neck area, where the mandible and maxilla are the. 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. This patient also has significant growth disturbance of the maxilla and may require subsequent. The continuing growth of embryo brings both the nasal placodes and the maxillary processes together in midline to form the maxilla and the beginning of the external nose. Orthodontic treatment of patient with maxillofacial fibrous dysplasia. Fibrous dysplasia fd is a sporadic benign skeletal disorder that can affect one bone monostotic.
Full text pdf 4016k abstracts references10 full text pdf. Get a printable copy pdf file of the complete article 1. Disease occurs along a broad clinical spectrum ranging from asymptomatic, incidental lesions, to severe disabling disease. However, lesions can develop in the skull, maxilla. An external file that holds a picture, illustration, etc. The involvement of the craniofacial skeleton is not uncommon and, generally, produces facial asymmetries. Surgery, particularly in a challenging region such as the skull base, should be reserved for patients with functional impairment or a cosmetic deformity. 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. A histopathological and radiological analysis with full clinical documentation.
Segmental odontomaxillary dysplasia is a painless, unilateral enlargement of the upper jaw. Metaphyseal dysplasiamaxillary hypoplasiabrachydacty syndrome is characterized by metaphyseal dysplasia associated with short stature and facial dysmorphism a beaked nose, short philtrum, thin lips, maxillary hypoplasia, dystrophic yellowish teeth and acral anomalies short fifth metacarpals andor short middle phalanges of fingers two and. In mfd, the zygomatico maxillary complex zmc most commonly involved in pfd and mas, the craniofacial region is involved in 90% of the cases and the anterior cranial base is involved in over 95% of cases. This study evaluates the possibility of diagnosing fibrous dysplasia. All archival cases diagnosed as fd and of from 19922006 were retrieved from the undh oral. Fibrous dysplasia genetic and rare diseases information. Fibro osseous lesion is a commonly used term that includes bone dysplasias, as well as neoplasms and other lesions of bone 1.
Patients may exhibit involvement of one bone monostotic fd. However it is not well understood why males tend to express the disease more frequently. Cherubism has also been found from the random mutation of a gene in an individual having no family history of the condition. 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 is a developmental anomaly of the skeletal system where an anomaly of the boneforming mesenchyme manifests as defects in osteoblastic differentiation and maturation. Fibrous dysplasia is a rare bone disorder characterized by the replacement of normal bone by abnormal fibrous tissue.
Fibrous dysplasia ossifying fibroma of the maxilla. It causes bone pain, deformities, and pathological fractures. Only cases which had a histological confirmation of fibrous dysplasia were included. We retrospectively studied the clinicopathological findings in 90 cases of fibrous dysplasia and 17 cases of osteofibrous dysplasia. Simple diagnostic approach for mandible and maxilla lesions. Fibrous dysplasia of the maxilla jama otolaryngology. Metaphyseal dysplasia maxillary hypoplasia brachydactyly.
As a result, most complications result from fracture, deformity, functional impairment, and pain. Diagnostic difficulties in fibrous dysplasia romanian journal of. Pdf maxillary fibrous dysplasia in a yearling filly. It may involve one bone or multiple bones polyostotic. Pdf objective fibrous dysplasia fd is a benign bone disorder in facial bones. Fibrous dysplasia of the maxilla, journal of pediatric. Fibrous dysplasia usually get stabilized after puberty 1. Maxillary fibrous dysplasia in a yearling filly article pdf available in the canadian veterinary journal.
Fibrous dysplasia is a benign skeletal disorder in. It is a nonhereditary disorder of unknown origin and is commonly seen in. University of nairobi dental teaching hospital undh. Regarding fibrous dysplasia regrowth, there is evidence of regrowth in patients 5 and 6. Fibrous dysplasia is a developmental tumor like condition that is characterized by. Fibrous dysplasia is a congenital developmental anomaly of bone which may occur in localised, regional or systemic forms. Fibrous dysplasia, maxilla, monostotic, polyostotic. This abnormality is a defect in the greater sphenoid wing and enlargement of the middle cranial fossa and was well. 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. In this condition the normal bone is replaced by immature fibro cellular tissue, which may be considered to be a fertile soil for a plethora of lesions like aneurysmal bone cyst,15 solitary bone cyst,6,7 and non specific cystic degeneration. Any information contained in this pdf file is automatically generated from digital material. Objective fibrous dysplasia fd is a benign bone disorder in facial bones.
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. Patient 5 has required subsequent limited resections in his 6 years of followup. A facial cleft is an opening or gap in the face, or a malformation of a part of the face. Here, we are presenting two case reports of fd involving the maxilla. Facial clefts is a collective term for all sorts of clefts. Modern imaging modalities and histopathologic analysis have made diagnosis relatively straightforward.
62 73 1381 415 645 500 1223 317 1520 1243 1500 389 1404 844 920 984 48 281 1055 1531 150 587 668 1197 1109 841 814 485 1001 419 1085 206 482 970 1238 313 393 352 454 457 46 19