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 adalah suatu jenis kelainan tulang dari proliferasi lesi. As these areas of fibrous tissue grow and expand over time, they can weaken the bonecausing it to fracture or become deformed. Fibrous dysplasia is a disease that causes growths or lesions in one or more bones of the human body. Pdf fibrous dysplasia of the bones is an uncommon congenital skeletal disorder that is found equally in both genders and is not inherited. Fd is a bone developmental anomaly characterized by replacement of normal bone and marrow bone by fibrous tissue. It may involve one bone or multiple bones polyostotic. Fibrous dysplasia represents about 2, 5% of all bone tumors and over 7% of all benign tumours. Disease occurs along a broad clinical spectrum ranging from asymptomatic, incidental lesions, to severe disabling disease. It often involves the long bones, craniofacial bones, ribs, and pelvis. The mutation occurs when a baby is developing in the womb.
In most cases, fibrous dysplasia occurs at a single site in one bone, but can occur at multiple sites in multiple bones. Monostotic fibrous dysplasia is the least complicated type of fibrous dysplasia. Fibrous dysplasia diagnosis and treatment mayo clinic. 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 fibrous dysplasia is a form of fibrous dysplasia affecting more than one bone. Fibrous dysplasia fd is a rare bony disorder in which normal bone is replaced by abnormal fibroosseous tissue. Users may be experiencing issues with displaying some pages on. Differential diagnoses includes osteofibrous dysplasia, osteochondroma. Osteofibrous dysplasia is a rare, benign nonneoplastic condition with no known cause. Fibrous dysplasia support groups online dailystrength.
Here a case of fibrous dysplasia of maxillary bone has reported. Fibrous dysplasia fd of bone is a rare bone disease leading to bone pain, fracture or bone deformity, and sometimes leading to neurological compression. The fibrous dysplasia is a benign bone disease, of slow growth and unknown etiology. Pfd is associated with bone pain and fractures due to bone fragility.
What are the signs and symptoms of fibrous dysplasia. It affects only one bone and most often occurs in the ribs, thigh bone, shin bone, or one of the facial bones. Polyostotic fibrous dysplasia is a rare disease, which was already described in 1938 by lichtenstein. Unduh sebagai doc, pdf, txt atau baca online dari scribd. 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. The involvement of the craniofacial skeleton is not uncommon and, generally, produces facial asymmetries. Fibrous dysplasia fd is a benign intramedullary fibroosseous lesion.
Fibrous dysplasia usually occurs in children ages 3 to 15, but it sometimes is not diagnosed until adulthood. 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. The etiology of fibrous dysplasia has been linked to an activating mutation in the gene that encodes the. Fibrous dysplasia adalah suatu kelainan tulang yang ditandai dengan penggantian tulang normal oleh jaringan ikat yang mengalami proliferasi. 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 is an uncommon bone disorder in which scarlike fibrous tissue develops in place of normal bone. These lesions are tumorlike growths that consist of replacement of the medullary bone with fibrous tissue, causing the expansion and weakening of the areas of bone involved. It involves any of the bones as single lesion monostotic or in multiple bone lesions polyostotic or all of the skeletal system panostotic. Fibrous dysplasia fd is a fibroosseous lesion with no apparent familial, hereditary or congenital basis. What is fibrous dysplasia childrens hospital colorado. Frequently, the skull bones, or long bones of the limbs are affected. Fibrous dysplasia is a common benign skeletal lesion that may involve one bone.
Most cases of craniofacial fibrous dysplasia cannot be truly categorized as monostotic because of the involvement of multiple adjacent bones of the craniofacial skeleton. Areas of healthy bone are replaced with this fibrous tissue. Fibrous dysplasia foundation helping people worldwide. The aim of this article is to represent a rare case of bilateral fibrous dysplasia of the upper and lower jaws, in combination with intellectual disability previously called mental retardation. The condition may be diagnosed, therefore, even in a person with no symptoms who is getting an xray for other reasons.
Polyostotic fibrous dysplasia pfd is a nonhereditary congenital and benign disease of the bone. 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. Generally there are no signs and symptoms, if the condition is mild. The primary tool for diagnosis of fibrous dysplasia is an xray. Scribd is the worlds largest social reading and publishing site. For the polyostotic form, the lesions often occur in younger patients and can involve numerous bones, sometimes more than half of the bones in the skeletal system. It is characterised by replacement of the normal bony architecture by abnormal bone which invariably leads to thickening of the bone.
Polyostotic fibrous dysplasia mccune albright syndrome. A comparative study of fibrous dysplasia and osteofibrous dysplasia with regard to expressions of cfos and cjun products and bone matrix proteins. Fibrous dysplasia has a varied radiographic appearance. Fibrous dysplasia is a disorder where bone is replaced by fibrous tissue. This irregular tissue can weaken the affected bone and cause it to deform or fracture. Fibrous dysplasia, maxilla, monostotic form, ground glass appearance. Fibrous dysplasia is an uncommon condition that is often characterized by the uneven growth of bones, pain, brittle bones, and bone deformity. As a result, most complications result from fracture, deformity, functional impairment, and pain.
Today we divide fibrous dysplasia into several forms and syndromes. The term craniofacial fibrous dysplasia cfd is used to describe fibrous dysplasia where the lesions are confined to contiguous bones of the craniofacial skeleton. Fibrous dysplasia is a benign disease in which medullary bone is replaced by fibroosseous tissue, and causes distortion and overgrowth of the involved bone and represents about 3% of all bone tumors. The replacement of normal bone in fibrous dysplasia can lead to pain, misshapen bones, and fracture, especially when it occurs in the long bones arms and legs. Fibrous dysplasia of bone and mccunealbright syndrome. Fibrous dysplasia is a condition characterized by abnormal bone growth.
Fibrous dysplasia is a benign fibroosseous lesion characterised by the replacement of normal bone by excessive proliferation of cellular fibrous connective. The fibrous dysplasia foundation serves people with fibrous dysplasia and mccunealbright syndrome fdmas, and related rare bone diseases like cherubism, by funding research, developing support networks, linking patients and doctors, dispersing news and. Introduction fibrous dysplasia fd is an uncommon developmental bone disease of benign origin. It is a benign fibroosseous disease process in which normal bone is replaced by fibrous tissue and immature bone. Campanacci described this condition in two leg bones, the tibia and fibula, and coined the term. Fibrous dysplasia overview nih osteoporosis and related. The exact cause of fibrous dysplasia is not known, but it is believed. In pfd, fibrouslike tissues with immature osteogenesis replace the normal bone.
Fibrous dysplasia genetic and rare diseases information. Fibrous dysplasia is a skeletal disorder in which boneforming cells fail to mature and produce too much fibrous, or connective, tissue. Fibrous dysplasia is a skeletal disorder that is characterized by the replacement of normal bone with fibrous bone tissue. Fibrous dysplasia differential diagnosis of cystic. Approximately 30% of monostotic fd mfd lesions are found in the cranial or facial bones. Fibrous dysplasia may involve one bone monostotic form or, less commonly, multiple bones polyostotic form. This is an enhanced pdf from the journal of bone and joint surgery. When this involves the skull, this can solely involve the skull bone or be associated with other bones in the body. Fibrous dysplasia fd is a sporadic benign skeletal disorder that can affect one bone monostotic form or multiple bones polyostotic form. Most people have symptoms by the time they are 30 years old. The treatment can be either conservative or complete resection. It is a nonneoplastic developmental hamartomatous disease of the bone, characterised by a blend of fibrous and osseous elements in the region.
A small proportion of patients also have endocrine abnormalities pdf file. 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. Fibrous dysplasia is linked to a problem with genes gene mutation that control boneproducing cells. This condition should be differentiated from nonossifying fibroma and fibrous dysplasia of. Polyostotic fibrous dysplasia mccunealbright syndrome. While bone appears solid in an xray, a fibrous dysplasia lesion has a relative distinct appearance often described as ground glass. We are working closely with our technical teams to resolve the issue.
Rib fibrous dysplasia expansile lytic lesion with no rib destruction. Pelvis fibrous dysplasia expansile, lytic, bubbly lesion. Fibrous dysplasia is a congenital, nonhereditary, progressive, skeletal disorder by which normal bone is replaced by a variable amount of structurally weak fibrous and osseous tissue. 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 is an abnormal condition of bone which can affect any of the bones in the body. Fibrous dysplasia is a benign noncancerous bone condition in which abnormal fibrous tissue develops in place of normal bone. The fibrous dysplasia foundation serves people with fibrous dysplasia and mccunealbright syndrome fdmas, and related rare bone diseases like cherubism, by funding research, developing support networks, linking patients and doctors, dispersing news and information for people affected by fibrous dysplasia. The gene for fd is located on band 20q, an area that codes for the. Monostotic fibrous dysplasia is a form of fibrous dysplasia where only one bone is involved.
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