Disorders of bone lab Slide 2 1- Bluish thin sclera and hyper mobile joints 2- Osteogenesis imperfecta 3- Mutations in the

genes that code for type I collagen 4 Thin translucent skin Distortion of ear ossicles and deafness Heart valve defects Dentinogenesis imperfecta Slide 3 1- Translucent teeth and obliterated pulps 2- Dentinogenesis imperfecta 3- Both conditions are carried on SEPARATE but related genes Slide 4 1 Increased density of the whole skeleton with no distinction between cortical and medullary bone The base of the skull shows marked radiopacity The density of jaw bones makes the roots of the teeth almost invisible on radiographs 2- Osteopetrosis (marble bone disease, stone bone disease) 3- Defect in osteoclastic activity and a failure in the proper remodeling of the

developing bone
4 Excessive density (quantity) of all bones with obliteration of marrow cavities Anemia Neutropenia and increased susceptibility to infections 5 Delayed eruption of teeth Osteomyelitis after extraction
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Slide 5
1- Patient can bring his two shoulders forward until they meet in the midline due to

partial or complete absence of the clavicles 2- Cleidocranial dysplasia 3- Mutations to the gene that encodes for differentiation of osteoblasts from osteogenic precursor cells 4 Skull Clavicle Jaws 5 The fontanelles and the sutures tend to remain open The skull appears flat with prominent frontal, parietal and occipital bones The nasal bridge is also depressed Slide 6 1 The deciduous dentition tends to be retained The permanent dentition tends to be delayed or non-eruptive because of multiple impactions Supernumerary teeth and dentigerous cysts The roots of the teeth tend to be thinner than normal Secondary cellular cementum is either absent or sparsely present on both deciduous and permanent teeth Slides 7 and 8 1234Monostotic fibrous dysplasia and it is related to fibro-osseous lesions Maxilla Craniofacial fibrous dysplasia Children and young adults
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5- Not diagnosed until adult life 6- Buccally 7- No, they respect suture lines and remain unilateral Slide 9 1- Left side of the patient ill-defined radiopaque obliteration of maxillary sinus and involvement of orbital floor 2- The lesions tend to expand mainly during the period of active skeletal growth and become quiescent in adult life treatment is delayed until phase of active growth subsides 3- COSMETIC SURGERY fibrous dysplasia is not radiosensitive and theres increased incidence of malignant transformation into fibrosarcoma among radiotherapy treated cases Slide 10 12345Ground glass or orange peel stippling appearance Ill-defined and gradually blend with surrounding bone Monostotic fibrous dysplasia 1 Radiopaque stage

Slide 11 1234Polystotic fibrous dysplasia Children and young adults, mainly females Diagnosed in childhood caf au lait melanotic spots

5- Serum alkaline phosphatase may be elevated depending on severity

6- McCune-Albright syndrome Other features: Precocious puberty in females puberty occurring at early age Other endocrine abnormalities Pigmentation of the oral mucosa
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Slide 12 1- Periapical cemental dysplasia 2- Cement osseous dysplasias are localized to the jaws and predominately involve the teeth bearing areas 3- Mandible 4- Affect whom is over 30 years of age, mainly females 5 Periapical granuloma Chronic periapical abscess Radicular cyst OKC Periapical cemental dysplasia ** Do pulp vitality testing and notice wither lesion is well or ill defined radiographically Slide 13 1- Fibrous tissue containing islands and trabeculae of woven bone What has happened? Replacement of old normal bone by fibrous tissue within which islands and trabeculae of new immature woven bone are built 2- 1 immature woven bone island or trabeculae (resembling Chinese characters) 2 fibrous cellular tissue 3- Fibro-osseous lesions Differential diagnosis: Fibrous dysplasia (monostotic and polystotic) Cemento-osseous dysplasia Ossifying fibroma 4- Clinically and radiographically BUT NOT histologically 5- At the margins of the lesion, if the lesional bone gradually fuses with that of the surrounding normal bone then it is dysplasia, otherwise it is neoplasia 6- With increasing age and maturity of the lesions the amount and cellularity of the fibrous tissue decreases while the amount of bone increases and there will be progressive remodeling of the woven bone into lamellar bone
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Jaw lesions differ from other long bone lesions in:

In jaw lesions, trabeculae may be thicker and blunter than in long bones Spherical areas of calcification resembling cementum may be present Osteoblastic and osteoclastic activity may be seen in relationship to some trabeculae

Slide 14 1- Cherubic appearance Eyes upturned to heaven Chubby face 2- Cherubism 3- Mutation in gene associated with fibroblast growth factor receptor 3 (FGFR3) 4- Mandible is always involved either alone or in combination with the maxilla 5- Males 6A Normal at birth and but painless bilateral swelling of the jaws appear between the ages of 2 and 4 years B Up to the age of 7 years C Regresses after 7 years of age with progressive reduction in asymmetry as patient passes from puberty into adult life 7- Cherubism is self-limiting condition and theres progressive improvement in facial appearance from about puberty onwards BUT conservative cosmetic surgery is often required to deal with residual deformity Slide 15 1- Well-defined bilateral multilocular radiolucencies at the angle and ramus of the mandible 2 Premature loss of deciduous teeth Displacement, lack of eruption, and failure of development of many permanent teeth Slide 16 1- Fibrous tissue containing various numbers of multinucleated giant cells What has happened?!
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Replacement of old normal bone by fibrous tissue within which varying amounts of multinucleated giant cells are found 21 multinucleated giant cell 2 fibrous cellular tissue 3- Giant cell lesions of bone Differential diagnosis: Cherubism Brown tumor of hyperparathyroidism Central giant cell granuloma Central giant cell tumor Peripheral giant cell granuloma

4- Clinically and radiographically BUT NOT histologically 5- As the lesion matures, activity decreases and it becomes progressively more fibrous, the number of giant cells diminishes and varying amounts of metaplastic bone are laid down Slide 17 and 18 1- Brown tumor of hyperparathyroidism 2- Biochemical investigation 3 Elevated parathormone level Increased serum calcium level Reduced serum phosphate level Increased urinary excretion of calcium and phosphate There may be elevated alkaline phosphatase 4- Middle aged women 5- Mandible 6 Bone pain Bone cysts (osteitis fibrosa cystica) Pathological fractures
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Brown tumors Renal colics due to stones Mental changes including depression, emotional liability and memory defects Increased incidence of peptic ulcer Chronic pancreatitis Hypertension

7- Biochemical investigation should be done to exclude brown tumor of hyperparathyroidism 8- Partial loss of lamina dura around the teeth may occur but it is NOT a constant feature Slide 19 1- Rickets 2- Vitamin D deficiency 3 Delayed eruption Enamel hypoplasia Increased width of Predentine Large amounts of interglobular dentine lack of vertical growth of the mandibular ramus

4- In osteoporosis the bone present is normally mineralized but reduced in density (quantity) In rickets the bone present is less mineralized Slide 20 1- Acromegaly 2- Excessive secretion of growth hormone after closure of epiphyseal plates 3- Spaced teeth and class III skeletal relationship Slide 21 1- Chronic Suppurative osteomyelitis 2- Irregular moth-eaten appearance 3- The source of the infection is usually a dental infection associated with adjacent teeth or a local trauma
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4- Mandible because: Thick trabeculae and cortical plates mandible has Blood supply is derived from an end artery which is the mandibular artery 5- Eventually a mass of necrotic bone (sequestrum) which is bathed in pus is formed and it becomes separated by osteoclastic activity from the surrounding vital bone The sequestrum may be spontaneously exfoliated through a sinus or have to be surgically removed to enhance healing Slide 22 1- Devitalized lamellar bone sequestrum with scalloped edges and absence of stainable osteocytes and osteoblasts and osteoclasts are seen all around Slide 23 1234Garres osteomyelitis (Chronic osteomyelitis with proliferative Periostitis) Onion-peel appearance Children and young adults Mandible

Slide 24

1- Focal sclerosing Osteitis (focal condensing osteitis)

2 Cemento osseous dysplasia Dense bone island Slide 25 1- Tori (Exostosis occurring at specific locations) 2- NO 3- Cosmetic reasons or denture construction 4 Rare in children Slow growth Benign outgrowth lesions of bone (not benign neoplasms) They are made of either dense cortical bone or Cancellous bone
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Slide 26 1- Dense bone Island Idiopathic osteosclerosis 2 Cemento-osseous dysplasia Focal sclerosing osteitis Osteoma Slide 27 1- Buccal exostosis 2- Osteoma Slide 28 1- Pagets disease of bone 2- Primary dysfunction of Osteoclasts leading to disorganized formation and remodeling of bone UNRELATED to functional requirements 3- Unknown BUT maybe paramyxovirus infection or genetic predisposition
4-

An initial predominantly osteoclastic phase An active stage of mixed osteolysis and osteogenesis A predominantly osteogenic phase 5 1st phase root resorption 2nd phase post extraction hemorrhage 3rd phase Hypercementosis, ankylosis, post extraction fracture and post extraction infections (osteomyelitis and dry socket) Slide 29 1- Reversal line 2nd active phase Mosaic appearance 2- Maxilla 3 varying degrees of bony deformity and distortion of weight bearing bones of the skeleton
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Enlargement of skull and facial bones Most cases are mild and the disease is discovered incidentally on radiographs Some patients complain of bone pain due to compression of nerves When the skull is involved patients may also present with signs and symptoms of sensory or motor disturbances related to narrowing of skull foramina Increased pathological fractures When the maxilla is involved and with progressive enlargement the alveolar ridge becomes thickened and widened, the palate flattened, and theres increasing facial deformity Slide 30 12345Cotton wool appearance (ill-defined irregular radiopaque areas) Loss of lamina dura, hypercementosis, Ankylosis, root resorption Osteosarcoma Biochemical investigations A- Normal B- Normal C- Raised Slide 31 1 Central giant cell granuloma Central giant cell tumor Brown tumor of hyperparathyroidism 2- Biochemical investigations (to exclude brown tumor) Slide 32 1- Peripheral Osteoma 2- Exostosis (when it is peripheral) and dense bone island (when it is central) 3- Gardner syndrome

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Manifestations are: Multiple osteomas of the jaws Polyposis coli which shows a marked tendency to undergo malignant changes Multiple fibrous tumors Epidermal/sebaceous cysts of the skin Multiple impacted supernumerary and permanent teeth may be found as well Slide 35 1- Osteosarcoma 2- Sun rays appearance only in 25% of patients and it can be seen in other malignancies of bone 3- Peripheral lesions (Juxtacortical lesions) and mandibular lesions Slide 36 1- Early feature of Osteosarcoma is localized symmetrical widening of the PDL space 2- The abnormal osteoid (malignant bone) and malignant osteoblasts Slide 37 1- Ossifying fibroma 2- Fibrous dysplasia and Cemento-osseous dysplasia Slide 38 1- Ossifying fibroma 2- Psammomatoid ossifying fibroma 3- Juvenile ossifying fibroma Features: Rapidly growing lesions affecting children and adolescents Histologically characterized by richly cellular mitotically active fibrous tissue with trabeculae of immature woven bone Must be distinguished from Osteosarcoma They carry a 30-60% recurrence rate
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Slide 39 1- Langerhans cell histiocytosis 2-

1 2
Slide 40

Eosinophil Malignant langerhans cell

1- Birbeck granules
2- Immunohistochemistry (Histiocytes also express surface antigens (CD-1a) shared by

normal Langerhans cells) Slide 41 1- Punched out appearance (Sharply demarcated round or oval osteolytic lesions) 2- Monoclonal (it is the result of neoplastic proliferation of a single clone of immunoglobulin producing cells (plasma cells) and is characterized by the production of large amounts of a single homogeneous type of immunoglobulin most commonly IgG) Slide 42 1- Malignant plasma cells 2- M-Spike (through protein electrophoresis of blood)
3-

Bence Johns proteins (urinary para-proteins composed of free light chains) identified in about 50% of patients' urine Hypercalcaemia Amyeloidosis Slide 44 1- Chondrosarcoma 2- Malignant chondroid and malignant chondroblasts

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