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Diseases of Bone manifested in the Jaws
 

Diseases of Bone manifested in the Jaws

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    Diseases of Bone manifested in the Jaws Diseases of Bone manifested in the Jaws Presentation Transcript

    • Diseases of Bone Manifested in the Jaws Dr. Anu Sushanth. A
    • • Bone dysplasias constitute a group of conditions in which normal bone is replaced with fibrous tissue containing abnormal bone or cementum.
    • FIBROUS DYSPLASIA • Fibrous dysplasia results from a localized change in normal bone metabolism that results in the replacement of all the components of cancellous bone by fibrous tissue containing varying amounts of abnormal-appearing bone. • At the histologic level, the result is the appearance of numerous short, irregularly shaped trabeculae of woven bone. • These trabeculae are not aligned in response to stress, but rather have a random orientation.
    • • Monostotic • Polyostotic – Jaffe lichtenstein – McCune-Albright syndrome • Craniofacial
    • Clinical Features • The solitary (monostotic) – 70% of all cases, – Most often involves the jaws. – The most common sites (in order) are the ribs, femur, tibia, maxilla, and mandible. – Discovered in a slightly older age group • Polyostotic – Children younger than 10 years, • The lesions usually become static when skeletal growth stops, but proliferation may continue, particularly in the polyostotic form.
    • The lesions may become active in pregnant women or with the use of oral contraceptives; and abnormal growth may occur after surgical intervention in young patients. No sexual predilection except for McCune-Albright syndrome, which affects females almost exclusively. Unilateral facial swelling or an enlarging deformity of the alveolar process. If extensive craniofacial lesions have impinged on nerve foramina, neurologic symptoms such as anosmia (loss of the sense of smell), deafness, or blindness may develop.
    • • Sarcomatous changes are unusual but have been reported, especially if therapeutic radiation has been given. • lesions within the area of fibrous dysplasia, such as aneurysmal bone cysts or giant cell granulomas
    • Radiographic Features • Location. – Fibrous dysplasia involves the maxilla almost twice as often as the mandible and occurs more frequently in the posterior aspect. – Lesions more commonly are unilateral.
    • • Periphery. • most commonly is ill defined, with a gradual blending of normal trabecular bone into an abnormal trabecular pattern. • Occasionally the boundary between normal bone and the lesion can appear sharp and even corticated, especially in young lesions
    • • The density and trabecular pattern of fibrous dysplasia lesions vary considerably. • The variation is more pronounced in the mandible and more homogeneous in the maxilla. • The internal aspect of bone may be more radiolucent, more radiopaque, or a mixture of these two variations compared with normal bone
    • Various trabacular patterns • The abnormal trabeculae usually are shorter, thinner, irregularly shaped, and more numerous than normal trabeculae are. • This creates a radiopaque pattern that can vary • ground-glass – appearance, resembling the small fragments of a shattered windshield • peau d ’ orange – A pattern resembling the surface of an orange • cotton wool – Wispy arrangement • Amorphous – Dense pattern • Fingerprint – Organization of the abnormal trabeculae into a swirling pattern
    • • Effects on Surrounding Structures. • Small lesions may have no effect • Expansion with maintenance of a thinned outer cortex
    • • May expand into the antrum by displacing its cortical boundary and subsequently occupying part or most of the maxillary sinus. • Often the extension into the sinuses appears as a parallel thickening of the outer cortical border, resulting in a residual antral air space that still has approximately the normal anatomic shape of a an antrum
    • • periodontal ligament space may appear to be very narrow. • Fibrous dysplasia can displace teeth or interfere with normal eruption, complicating orthodontic therapy. • Fibrous dysplasia appears to be unique in its ability to displace the inferior alveolar nerve canal in a superior direction
    • Differential Diagnosis • 1. Hyperparathyroidism – These diseases are polyostotic; bilateral; and, unlike fibrous dysplasia, do not cause bone expansion. • 2. Paget ’ s disease – It occurs in an older age group, and when it involves the mandible, the whole mandible is involved, • 3. Periapical cemental dysplasia – it often is bilateral, with an epicenter in the periapical region. – also occurs in an older age group
    • • Osteomyelitis: • may result in enlargement of the jaws, but the additional bone is generated by the periosteum; therefore the new bone is laid down on the surface of the outer cortex, and close examination may reveal evidence of the original cortex within the expanded portion of the jaw. • The identification of sequestra aids in the identification of osteomyelitis.
    • OSSEOUS DYSPLASIAS Periapical Cemental Dysplasia Florid Osseous Dysplasia
    • Periapical Cemental (osseous) Dysplasia • Periapical cemental dysplasia (PCD) is a localized change in normal bone metabolism that results in the replacement of the components of normal cancellous bone with firous tissue and cementum-like material, abnormal bone (similar to that seen in fibrous dysplasia), or a mixture of the two • Clinical Features • middle age (39) • nine times more often in females • The involved teeth are vital, • and the patient usually has no history of pain or sensitivity. • incidental finding • The lesions can become quite large, causing a notable expansion
    • Radiographic Features • Location • The epicenter of a PCD lesion usually lies at the apex of a tooth • predilection for the mandibular anterior teeth, • In most cases the lesion is multiple and bilateral, but occasionally a solitary lesion arises
    • Periphery and shape • Well defined • Overall round or oval shape centered over the apex of the tooth • Often a radiolucent border of varying width is present, surrounded by a band of sclerotic bone that also can vary in width
    • • The internal structure varies, depending on the maturity of the lesion. • 3 stages • In the early stage, normal bone is resorbed and replaced with fibrous tissue that usually is continuous with the periodontal ligament (causing loss of the lamina dura)
    • • In the mixed stage, radiopaque tissue appears in the radiolucent structure. • This material usually is amorphous; has a round, oval, or irregular shape; and is composed of cementum or abnormal bone
    • • In the mature stage, the internal aspect may be totally radiopaque without any obvious pattern. • Usually, a thin radiolucent margin can be seen at the periphery because this lesion matures from the center outward • simple bone cysts form within the cemental lesions
    • • Effects on Surrounding Structures • The normal lamina dura of the teeth involved with the lesion is lost, making the periodontal ligament space either less apparent or giving it a wider appearance • although in rare cases some root resorption may occur. • Some lesions stimulate a sclerotic bone reaction from the surrounding bone.
    • Differential Diagnosis • Periapical abscess/granuloma – Teeth are vital in case of POD • benign cementoblastoma (for mature lesion) – This tumor is usually is attached to the surface of the root, which may be partly resorbed
    • Florid Osseous Dysplasia • Florid osseous dysplasia (FOD) is a widespread form of POD • Normal cancellous bone is replaced with dense acellular cemento-osseous tissue in a background of fibrous connective tissue. • The lesion has a poor vascular supply, a condition that likely contributes to its susceptibility to infection. • In some cases a familial trend can be seen. • No clear definition indicates when multiple regions of PCD should be termed FOD. • “However, if PCD is identified in three or four quadrants or is extensive throughout one jaw, it usually is considered to be FOD”
    • Clinical Features • female and middle aged (mean age, 42 years) • Occasionally patients complain of low-grade, intermittent, poorly localized pain in the affected bone, especially when a simple bone cyst has developed within the lesion • If the lesions become secondarily infected, features of osteomyelitis may develop, including mucosal ulceration, fistulous tracts with suppuration, and pain.
    • Radiographic Features • Location. • FOD lesions usually are bilateral and present in both jaws • The epicenter is apical to the teeth, within the alveolar process and usually posterior to the cuspid • lesions occur above the inferior alveolar canal
    • • Internal Structure • May be radiolucent, radiopaque, mixed. • Some prominent radiolucent regions, which usually represent the development of a simple bone cyst, may be present • The radiopaque regions can vary from small oval and circular regions (cotton-wool appearance) to large, irregular, amorphous areas of calcification.
    • • Effects on Surrounding Structures • Large FOD lesions can displace the inferior alveolar nerve canal, floor of the antrum, buccal and lingual cortical plates. • The roots of associated teeth may have a considerable amount of hypercementosis, which may fuse with the abnormal surrounding cemental tissue of the lesion.
    • Differential Diagnosis • Paget ’ s disease: – May also show cotton wool – type radiopaque regions with associated hypercementosis. – However, Paget ’ s disease affects the bone of the entire mandible, whereas FOD is centered above the inferior alveolar canal. – Furthermore, Paget ’ s disease often is polyostotic • chronic sclerosing osteomyelitis: – The cemental-like masses that are secondarily infected have a wider and more profound radiolucent border
    • Ossifying Fibroma • Ossifying fibroma is classified as and behaves like a benign bone neoplasm. • Juvenile ossifying fibroma is a very aggressive form of cof that occurs in the first two decades of life • Can vary from indolent to aggressive behavior. • Occur at any age but usually is found in young adults. • Females are affected more often than males. • Displacement of the teeth may be an early clinical • feature, although most lesions are discovered during routine dental examinations. • In cases of juvenile ossifying fibroma, rapid growth may occur in a young patient, resulting in deformity of the involved jaw.
    • Radiographic Features • typically inferior to the premolars and molars and superior to the inferior alveolar canal. • In the maxilla it occurs most often in the canine fossa and zygomatic arch area • The borders of COF lesions usually are well defined. • A thin, radiolucent line, representing a fibrous capsule, may separateit from surrounding bone
    • • Internal Structure – Mixed radiolucent- radiopaque – May appear almost totally radiolucent with just a hint of calcified material – Wispy (similar to stretched tufts of cotton) – Flocculent pattern (similar to large, heavy snowflakes) may be seen – Granular pattern similar to fibrous dysplasia • Effects on Surrounding Structures. • Outward expansion approximately equal in all directions, just like a tumor. • displacement of teeth or of the inferior alveolar canal and expansion of the outer cortical plates of bone. • outer cortical plate, although displaced and thinned, remains intact
    • • lamina dura of involved teeth usually is missing, and resorption of teeth may occur. Flocculant Solid Granular
    • Differential diagnosis • fibrous dysplasia: • The boundaries of a OF lesion usually are better defined • The internal structure of fibrous dysplasia lesions in the maxilla may be more homogeneous • Both types of lesions can displace teeth, but COF displaces from a specific point or epicenter • Fibrous dysplasia usually displaces the lateral wall of the maxilla into the maxillary antrum, maintaining the outer shape of the wall, whereas an ossifying fi broma has a more convex shape because it extends into the maxillary antrum • Tumor like concentric expansion
    • Ossifying fibroma Fibrous dysplasia
    • Differential diagnosis • Osseous Dysplasia: – cemental dysplasia usually is multifocal where as COF is not. – presence of a simple bone cyst is a characteristic of cemental dysplasia. – A wide sclerotic border and undulating expansion are more characteristic of the slow-growing cemental dysplasia – Tumor like concentric expansion
    • Central Giant Cell Granuloma • Central giant cell granuloma (CGCG) is thought to be a reactive lesion to an as-yet- unknown stimulus and not a neoplastic lesion • The histologic appearance consists primarily of fibroblasts, numerous vascular channels, multinucleated giant cells, and macrophages. • adolescents and young adults; at least 60% of cases occur in individuals younger than 20 years • The lesion usually grows slowly, although it may grow rapidly, creating the suspicion of a malignancy.
    • Radiographic Features Location. •Mandi •anterior to molars •Max •anterior to cuspids Younger individuals, •Posterior region Older individuals
    • Periphery. Mandi • usually produces a well- defined radiographic margin • no evidence of cortication Max • ill-defined, almost malignant-appearing
    • • Internal Structure – Small CGCG lesions show no evidence of internal structure. – A subtle granular pattern of calcification – Occasionally bone is organized into ill-defined, wispy septa they emanate at right angles from the periphery of the lesion. – a small indentation of the expanded cortical margin is seen at the point where this right-angled septa originate
    • Differential Diagnosis • ameloblastoma, – ameloblastomas tend to occur in an older age group and – more often in the posterior mandible, and – ameloblastomas have coarse, curved, well- defined trabeculae • odontogenic myxoma, – older age group, – may have sharper and straighter septa, – and do nothave the same propensity to expand as do giant cell granulomas • aneurysmal bone cyst. – comparatively rare – occur more often in the posterior aspect of the jaws and – usually cause profound expansion
    • Aneurysmal Bone Cyst • An aneurysmal bone cyst (ABC) usually is considered to be a reactive lesion of bone rather than a cyst or true neoplasm. • Some believe that it represents an exaggerated localized proliferative response of vascular tissue in bone. • Similar to the CGCG in both the radiographic and histologic appearance • Occasionally develop in association with other primary lesions such as fi brous dysplasia, central hemangioma, giant cell granuloma, and osteosarcoma. • More than 90% of reported jaw lesions have occurred in individuals younger than 30 years (females). • Usually manifests as a fairly rapid bony swelling
    • • Mandible :maxilla (3 : 2), and • Molar and ramus regions are more involved • Well defined, and the shape is circular or “ hydraulic. ” • Internal structure very similar to CGCG.
    • Differential Diagnosis • CGCG • Ameloblastoma
    • Paget ’ s Disease • Paget ’ s disease is a skeletal disorder and essentially a disease involving osteoclasts, resulting in abnormal resorption and apposition of osseous tissue in one or more bones. • involve many bones simultaneously, • It is initiated by an intense wave of osteoclastic activity, with resorption of normal bone resulting in irregularly shaped resorption cavities. • After a period of time, vigorous osteoblastic activity ensues, forming woven bone.
    • primarily a disease of later middle and old age. Males more than 65 years Affected bone is enlarged and commonly deformed, resulting in bowing of the legs, curvature of the spine, and enlargement of the skull. The jaws also enlarge when affected. Separation and movement of teeth may occur, causing malocclusion. Dentures may be tight or may fit poorly in edentulous patients.
    • Bone pain is an inconsistent symptom, most often directed toward the weight-bearing bones. Patients with Paget ’ s disease may also have ill-defined neurologic pain as the result of bone impingement on foramina and nerve canals. Patients with Paget ’ s disease often have severely elevated levels of serum alkaline phosphatase (greater than with any other disorder) during osteo
    • Radiographic Features Most often in the pelvis, femur, skull, and vertebrae and infrequently in the jaws Maxilla about twice as often as the mandible Whenever the jaws are involved, it is important to note that the entire mandible or maxilla is affected. Although bilateral, the involvement is asymmetrical
    • 3 stages •an early radiolucent resorptive stage, a granular •ground glass – appearing second stage, and •denser, more radiopaque appositional late stage. Trabecular patterns •1. linear pattern: The trabeculae may be long and may align themselves linearly •2. granular pattern: Numerous, short, with random orientation •3. cotton-wool appearance: trabeculae may be organized into rounded, radiopaque patches of abnormal bone, •The overall density of the jaws may decrease or increase, depending on the number of trabeculae
    • Effects on Surrounding Structures •Paget ’ s disease always enlarges an affected bone to some extent, •In enlarged jaws the outer cortex may be thinned but remains intact. •The outer cortex may appear to be laminated in occlusal projections. •Cortical boundaries such as the sinus floor may be more granular and less apparent as sharp boundaries. •The lamina dura may become less evident. •Often hypercementosis ( exuberant and irregular)develops on a few or most of the teeth in the involved jaw
    • Differential Diagnosis Fibrous dysplasia: • Paget ’ s disease occurs in an older age group • Always Bilateral • fibrous dysplasia has a tendency to encroach on the antral air space, whereas Paget ’ s disease does not. • The linear trabeculae and cotton-wool appearance of Paget ’ s disease are distinctive. Osseous dysplasia • these lesions are centered above the inferior alveolar nerve canal and • most commonly have a radiolucent capsule Metabolic bone disease • Paget ’ s disease enlarges bone, and metabolic diseases do not.
    • Cherubism • Cherubism is a rare inherited autosomal dominant disease that causes bilateral enlargement of the jaws, giving the child a cherubic facial appearance. • It is composed of giant cell granuloma – like tissue and • These lesions regress with age.
    • • Cherubism develops in early childhood between 2 and 6 years of age • The most common presenting sign is a painless, firm, bilateral enlargement of the lower face. • Because children ’ s faces are rather chubby, mild cases may go undetected until the second decade.
    • • Profound swelling of the maxilla may result in stretching of the skin of the cheeks, which depresses the lower eyelids, exposing a thin line of sclera and causing an “ eyes raised to heaven” appearance.
    • Radiographic Features • Location. • This lesion is bilateral and often affects both jaws. • When it is present in only one jaw, the mandible is the most common location. • The epicenter is always in the posterior aspect of the jaws, in the ramus of the mandible or the tuberosity of the maxilla
    • Periphery. • The periphery usually is well defined and in some instances corticated Internal Structure • The internal structure resembles that of CGCG, with fine, granular bone and wispy trabeculae forming a prominent multilocular pattern.
    • Effects on Surrounding Structures • Expansion of the cortical boundaries of the maxilla and mandible. • Maxillary lesions enlarge into the maxillary sinuses. • Because the epicenter is in the posterior aspect of the jaws, the teeth are displaced in an anterior direction. • The degree of displacement can be severe, and with some lesions the tooth buds are destroyed.
    • Differential Diagnosis • Multiple odontogenic keratocysts in basal cell nevus syndrome • Giant cell granuloma • Fibrous dysplasia
    • Langerhans ’ Cell Histiocytosis (Histiocytosis X,) Result from the abnormal proliferation of langerhans cells or their precursors. Langerhan’s cells are specialized cells of the histiocytic cell line that normally are found in the skin. The abnormal proliferation of langerhans cells and eosinophils results in a spectrum of clinical diseases.
    • Activation of histiocytes may be locally or systemically driven. Although the etiology is unknown, Immunologic defects and viruses have been implicated to cause the loss of control for proliferation. There is no proof that LCD is a malignancy. LCD may produce multiple lytic lesions affecting several bones at the same time.
    • • Eosinophilic granuloma (solitary/Chronic localized LCD), • Hand-Schüller-Christian disease (chronic disseminated LCD), • Letterer-Siwe disease (acute disseminated LCD).
    • Clinical Features • Head and neck lesions are common at initial presentation, • approximately 10% of all patients with LCH have oral lesions. • Often the oral changes are the first clinical signs of the disease.
    • Chronic Localized Eosinophilic granuloma (EG) usually appears in the skeleton (ribs, pelvis, long bones, skull, and jaws) and in rare cases in soft tissue. This condition occurs most often in older children and young adults but may develop later in life. The lesions often form quickly and may cause a dull, steady pain. In the jaws the disease may cause bony swelling, a soft tissue mass, gingivitis, bleeding gingiva, pain, and ulceration. Loosening or sloughing of the teeth often occurs after destruction of alveolar bone by one or more foci of EG
    • Chronic disseminated • Affects children and young adults. • form may involve multiple bone lesions, diabetes insipidus, and exophthalmos, (Hand- Schüller-Christian disease.) • Lymphadenopathy, splenomegaly, hepatomegaly. • Bone marrow suppression (Pancytopenia), due to excessive proliferation of Histiocytes.
    • Acute disseminated • Letterer-Siwe disease is a malignant form of LCH that most often occurs in infants less than 3 years of age. • Soft tissue and bony granulomatous reactions disseminate throughout the body, and the condition is marked by intermittent fever, hepatosplenomegaly, anemia, lymphadenopathy, hemorrhage, and failure to thrive. • Lesions in bone are rare. • Death usually occurs within several weeks of the onset of the disease.
    • Radiographic Features • The mandible is a more common site than the maxilla, and the posterior regions are more involved than are the anterior regions. • The mandibular ramus is a common site of intraosseous lesions. • Solitary lesions of the jaws may be accompanied by lesions in other bones
    • • The periphery of EG lesions varies from moderately to well defined but without cortication; the periphery sometimes appears punched out. • The bone destruction progresses in a circular shape. Hence when skeleton is involved, lesions are seen as multiple, rounded, radiolucencies. (Punched out)
    • In the jaw, bone destruction usually starts at mid-root region, after it includes a portion of the superior border of the alveolar process, it may give the impression that a section of the alveolar process has been scooped out • The internal structure usually is totally radiolucent. • Loss of Lamina Dura • Floating teeth appearance • Periosteal new bone reaction