If this happens, it can eventually lead to deformation of facial features and affect the. 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. Dentin dysplasia, type 1 genetic and rare diseases. Monostotic fibrous dysplasia with this type only one bone is affected and accounts for approximately seventy percent of all cases of fibrous dysplasia. 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. Fibrous dysplasia of the maxilla and mandible sciencedirect. There is no consensus regarding the clinical and radiographic findings present in patients with dentin dysplasia type i 6. 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 fibroosseous lesion with no apparent familial, hereditary or congenital basis. In most cases, fibrous dysplasia occurs at a single site in one bone, but can occur at multiple sites in multiple bones.
Fibrous dysplasia fd is a rare benign but progressive bone disorder. Fibrous dysplasia fd is a sporadic benign skeletal disorder that can affect one. Fibrous dysplasia and ossifying fibroma of the paranasal. It is a nonneoplastic developmental hamartomatous disease of the bone, characterised by a blend of fibrous and osseous elements in the region. 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.
Dentin dysplasia type ii nord national organization for. The cause is unknown, and the disease affects the jaws, teeth, and adjacent soft tissue. Segmental odontomaxillary dysplasia sod is an uncommon, nonhereditary, recently recognized developmental disorder affecting the upper jaw and related dental components. Any information contained in this pdf file is automatically generated from digital. The purpose of this study was to report six cases of fibrous dysplasia associated with the maxillary sinus. Radiographic appearance of fibrous dysplasia associated. Patients with cleft lip and palate and maxillary hypoplasia usually present with a. Fibro osseous lesions classifications, pathophysiology. Fibrous dysplasia fd and ossifying fibroma are the most common fibroosseous lesions. Different clinical presentation and management of temporal bone fibrous. Fibrous connective tissue containing abnormal bone replaces normal bone. The areas of fibrous tissue are interwoven with newly formed bone trabeculae that vary in size and shape. Radiological sequelae of developmental dysplasia of the hip epos.
Fibrous dysplasia is a benign intramedullary fibroosseous lesion originally described by lichtenstein1 in 1938 and by lichtenstein and jaffe in 19422. Developmental dysplasia of the hip ddh sydney childrens. In this case, the changes observed are not characteristic of any dd i subtype described 6,9,10. Only those reports of fd which occurred in a series in the reporting authors caseload were considered. Fibrous dysplasia involving the skull base and temporal bone. The frontonasal process is ectodermally derived, and it develops independently over the forebrain giving rise to the forehead and the nasal olfactory placodes. Fibrous dysplasia of maxillary sinus article pdf available in international archives of otorhinolaryngology 2 june 2009 with 58 reads how we measure reads. Dislocated the femoral head is completely outside the acetabulum. Radiographic analysis shows obliteration of all pulp chambers, short, blunted and malformed or absent roots and periapical radiolucencies of. Cof is more common in the mandible, tends to occur in anterior regions and is smaller in size, whereas fibrous dysplasia is more common in the posterior maxilla, and the lesions tend to be larger. In fibrous dysplasia there are characteristic changes in the blood chemistry foci of cartilage are a common histological finding. If you have problems viewing pdf files, download the latest version of adobe reader for language access assistance, contact the ncats public information officer genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 tollfree. Pdf fibrous dysplasia is a nonneoplastic skeletal disorder of unknown. Generally, the occurrence rate is higher in the maxilla than in the mandible.
Distraction osteogenesis do is an effective method used for bone regeneration. Fibrous dysplasia of the right maxillary bone tamer w kassem department of diagnostic and interventional radiology, university of cairo, egypt abstract fibrous dysplasia is a rare bone disease caused by mutations in gnas gene. 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. Rib fibrous dysplasia expansile lytic lesion with no rib destruction.
It can occur in the bones in the front of the head andor sphenoid bones that are situated at the base of the skull. It can also be associated with cleft lip and cleft palate. 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 is a localized disorder of bone characterized by abnormal proliferation of fibrous tissue. 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 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. Teratologic hip dysplasia refers to prenatal severe fixed dislocation usually. Fibrous dysplasia is usually a slowly progressive, benign disease that develops over several years and presents with deformity or mild symptomatology. The clinical behaviour and radiological findings of these cases were studied from the case files. Dentin dysplasia is a rare hereditary disturbance of dentin formation characterized by defective dentin development with clinically normal appearing crowns, severe hypermobility of teeth and spontaneous dental abscesses or cysts. The radiographic appearance of the irregularly shaped trabeculae aids in the differential diagnosis. Any information contained in this pdf file is automatically generated from digital material submitted to epos by third parties in the form of scientific presentations. Fibrous dysplasia of the right maxillary sinus, displacing the floor of the orbit and causing proptosis of the right eye.
There are two forms of this medical condition which are. It is characterized by abnormal development dysplasia of dentin. Background fibroosseous lesions are a group of processes characterized by replacement of normal bone by fibrous tissue containing a newly formed mineralized product. In mfd, the zygomaticomaxillary 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. Dysplasia, monostotic fibrous dysplasia, fibro osseous lesion is a commonly used term that includes bone dysplasias, as well as neoplasms and other lesions of bone. Fibrous dysplasia is a relatively rare tumorous lesion in the maxillofacial region. Fibrous dysplasia is a rare bone disease that represents 2. Fibrous dysplasia fd is a benign but chronic bone lesion characterized by the progressive replacement of normal bone with fibroosseous connective tissue. Axial bone window bone thickening and homogeneous ground glass appearance involving the right mandibular condyle, the squamous part and mandibular fossa of the right temporal bone which shows a. Four patients had surgery involving maxilla or mandible. Dentin dysplasia type ii, also known as coronal dentin dysplasia, is a rare genetic disorder that affects the teeth.
The databases searched were the pubmed interface of medline and lilacs. Fibrous dysplasia is a developmental tumor like condition that is characterized by. We describe a case of a young adult male with cementoossifying fibroma of paranasal sinus presenting acutely as left orbital cellulitis with proptosis. 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. There are many treatment option available, but still management of fd remains challenging. Fibrous dysplasia has 4 different disease patterns monostotic 70%. Fibrous dysplasia fd is a nonneoplastic, expansile lesion of unknown origin. Clinical guidelines for the management of craniofacial. Fibrous dysplasia treatment, symptoms, causes, pictures.
Pictures of fibrous dysplasia of the paranasal sinuses and skull. The true incidence and prevalence of fibrous dysplasia are difficult to estimate, but the lesions are not rare. Dentin is the hard tissue found beneath the enamel that surrounds and. Segmental odontomaxillary dysplasia sod is an uncommon disorder that causes a unilateral development of disturbance of the maxilla associated with an abnormal growth and maturation of bone, teeth, and adjacent gingival tissue, with or without facial cutaneous lesions. The phenotype is variable and may be isolated to a. Pelvis fibrous dysplasia expansile, lytic, bubbly lesion. Case report a 63 year old gentleman presented to clinic with. Screening programmes for developmental dysplasia of the hip in. Different clinical presentation and management of temporal bone. The radiographic appearance of this lesion varies widely in the jaw. In the facial area, it is more frequently found in. Aggressive fibrous dysplasia of the maxillary sinus. It generally has affinity for maxilla over mandible in craniofacial presentation. Oral rehabilitation by dental implants for teeth involved.
The normal bone is replaced with fibrous tissue and abnormal woven bone. Fibrous dysplasia is a bone disorder of unknown origin characterized by slow. Polyostotic fibrous dysplasia with this type it affects several bones and is often associated with mccunealbright syndrome, which is a. In about onefourth of cases this disorder affects the head and neck area, where the mandible and maxilla are the. Risk factors for hip dysplasia include a family history of a similar problem and female infants delivered in the breech position. It is a rare condition of uncertain etiology that results in painless unilateral expansion of the posterior dentoalveolar complex, gingival hyperplasia, lack of one or both premolars in the affected area. 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.
Fibrous dysplasia is an uncommon bone disorder in which scarlike fibrous tissue develops in place of normal bone. Distraction osteogenesis in the treatment of maxillary. Subramaniam and herle 368 fibrous dysplasia of the maxillary sinus. The premolars may be congenitally missing, and the primary teeth baby teeth may be smaller than usual. It causes bone pain, deformities, and pathological fractures.
Fibrous dysplasia and ossifying fibroma of the paranasal sinuses volume 112 issue 10 d. In 36, 3% of the cases of fd the clinical beginning is hidden, there is. Developmental dysplasia of the hip ddh is a condition in which the femoral head has an abnormal relationship to the acetabulum. Fibrous dysplasia is a skeletal developmental anomaly of the bone forming mesenchyme that manifest as defect in osteoblastic differentiation and maturation. Clinical and radiographic features of chronic monostotic. Pdf monostotic fibrous dysplasia affecting mandible. It is associated with crouzon syndrome, angelman syndrome, as well as fetal alcohol syndrome. Discussion fibrous dysplasia of the cranium is a rare disorder of unknown etiology in which normal bone is replaced by abnormal fibroconnective tissue proliferation.
Segmental odontomaxillary dysplasia is a painless, unilateral enlargement of the upper jaw. Advancement of the maxilla by use of rigid external distraction red device has been performed successfully and many other internal devices have been introduced for better results regarding the patients comfort. This irregular tissue can weaken the affected bone and cause it to deform or fracture. Single upper central incisor genetic and rare diseases. The groundglass matrix of the bone is highly suggestive of fibrous dysplasia. Maxillary hypoplasia, or maxillary deficiency, is an underdevelopment of the bones of the upper jaw, which produces midfacial retrusion and creates the illusion of protuberance jutting forward of the lower jaw.
89 734 1357 1264 747 920 1189 675 1410 716 507 1523 796 232 906 1061 307 511 1327 695 1157 403 186 1366 1458 1536 55 1010 1566 277 636 1319 269 1129 510 33 1091 1121 462 1389 490 1289 588 916 749