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Metabolic Bone Diseases.pptx

Apr 03, 2018

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Jk Flores
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    Rickets or osteomalacia Vitamin D deficiency

    Hypophosphatemia

    Hyperparathyroidism Primary hyperparathyroidism

    Secondary hyperparathyroidism

    Renal osteodystrophy Pagets Disease

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    Osteomalacia is caused by deficientcalcification of an otherwise

    normal protein bone matrix

    Due to failure of calcium and phosphorus

    deposition in bone matrix X-ray Findings:

    Involvement of the pelvis and long bones, withdemineralization and bowing

    Less often, the spine and skull are involved as well. Fractures are rare except for "pseudo fractures."

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    lucent band of decreased cortical density

    perpendicular to bone surface

    often multiple, +/- symmetrical +/- callus formation

    "pathognomonic" ofosteomalacia seen on compressive (concave) side (eg, prox

    femur) also seen in Paget disease on tensile (convex)

    side

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    AP view loosers zone frogleg view loosers zone

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    This is a classic pseudofractureand is pathognomonic for

    osteomalacia.

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    The skeletal effects are due to lack ofcalcification of osteoid.

    The most obvious change are at 'metaphysis'-where rapid growth is occurring. First change to appear is a 'loss of normal zone

    of provisional calcification' adjacent tometaphysis. This begins as an indistinctness of the

    metaphyseal margin, progressing to a 'frayed'appearance with widening of the growth plate,

    due to lack of calcification of metaphyseal bone. Weight bearing & stress on uncalcified bonegives rise to 'splaying' & 'cupping' ofmetaphysis.

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    A similar but less marked effect occurs insubperiosteal layer , which may cause lack ofdistinctness of cortical margin.

    Eventually a generalized reduction in bonedensity is seen. In the epiphysis - there may be somehaziness of cortical margin. Thus all findings

    in Rickets occur due to failure of calcification& abnormal demineralization

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    The first changes of rickets appear in rapidlygrowing distal ends of ulna & radius (wrist &knee are commonly involved due to moreuse).

    Rarefaction of provisional zone ofcalcification with widening of epiphysis-diaphysis distance is first to appear.

    Following treatment there is ossification ofprovisional zone of calcification.

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    Splaying, fraying, cupping, widening of growthplates of bilateral distal radius and ulna.

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    This x-ray shows a widened growthplate with an irregular pattern on the

    metaphyseal side.

    This is an x-ray of the same patient takenafter treatment with calcium and vitamin D.The growth plate has been reduced to itsnormal width as the cartilage has begun tocalcify. The margin is smooth and clean onthe end of the ulna. The calcification bands

    are visible on both radius and ulna.

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    This is an x-ray of a child withbowed legs due to rickets

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    Xrays:sub-periosteal

    resorption

    pepper pot skull/salt & pepperskull

    rugger jersey spine

    cystic brown tumours

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    Subperiosteal resorption of cortical bone isvirtually diagnostic of hyperparathyroid bone

    disease. In this form, a lacelike appearance of the

    phalangeal bone may progress to a spiculatedcontour and, eventually, to complete resorptionof the entire cortex.

    Other sites of subperiosteal resorption includethe phalangeal tufts; medial aspect of theproximal ends of the tibia, humerus and femur;superior and inferior margins of the ribs; and

    lamina dura. Subperiosteal resorption of bone may also be

    apparent at the margins of certain joints,especially those in the hand, wrist and foot

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    -The plain x-ray of the hand shows typical changes ofhyperparathyroidism, with resorption of subperiosteal bone on theradial side of proximal and middle phalanges of the 2nd and 3rd digits.-This is most obvious in the proximal phalanx of the middle finger.

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    X-ray Wrist shows marked subperiosteal bone resorption

    in the radial aspect of the middle phalanges of the 2nd

    and3rd digits.

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    X-ray Skull (Lateral) shows diffuse trabecular resorption,giving a granular 'salt and pepper' appearance. Loss of

    distinction between the outer and inner tables of theskull is seen

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    Xrays:sub-periosteal resorption

    pepper pot skull/salt &pepper skull

    rugger jersey spinecystic brown tumours

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    Xrays:sub-periosteal

    resorption

    pepper pot skull/salt &pepper skull

    rugger jersey spine

    cystic brown tumours

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    Xrays:sub-periosteal resorption

    pepper pot skull/salt &pepper skull

    rugger jersey spine

    cystic brown tumours

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    Xray appearance holes in bonewithout sclerotic reaction

    no reaction of the host bone to the lesions

    Bone scan may be negative

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    Radiology

    cortical thickening

    osteolytic, osteosclerotic and mixedlesions

    osteoporosis circumscripta

    bone scan

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    In the skull, the initial osteolytic phase of pagetsis called "osteoporosis circumscripta" due to thepresence of geographic lucent regions primarilywithin the frontal bone.

    This is followed by excessive new bone formation

    leading to cortical thickening and osteoscleroticchanges in the inner table, diploe, andsubsequently the inner surface of the outertable. In this phase, plain radiographs manifest a

    characteristic "cotton wool" appearance ofsclerotic lesions surrounded by areas ofdemineralization.

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    typical appearance of the skull inadvanced pagets

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    Characteristic x-ray findings include thefollowing: Increased bone sclerosis

    Abnormal architecture with coarse cortical

    trabeculation or cortical thickening Bowing

    Bony enlargement

    There may be stress microfractures of the tibia or

    femur.

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    This lateral skull view shows thetypical "cotton-wool" appearance.

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    Lateral radiograph of the skull shows adiffuse lucency affecting the frontaland temporal bones. The marginbetween lucent and normal bone iswell defined.

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    Paget's disease of the lumbar spine.Typical features include expansion ofbone, the picture frame appearance,obvious softening of bone, plasticity,and posterior element involvement.

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    Osteoporosis is caused by a deficiency oforganic bone matrix.

    Osteoporosis is a disease characterized bylow bone mass and loss of bone tissue thatmay lead to weak and fragile bones.

    Has an increased risk for fractured bones

    (broken bones).

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    The serum calcuim, phosphorus, and alkalinephosphatase are normal.

    Common causes ofgeneralized osteoporosis: Senility

    Cushings disease Hyperparathyroidism

    Severe childhood anemias

    Osteogenesis imperfecta

    Multiple myeloma

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    Local osteoporosis can result from: Disuse

    Neurovascular disturbances (Sudecks atrophy)

    Local joint disease

    * Osteoporosis is most marked in the spine and pelvis.

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    The earliest feature of spinalosteoporosis is prominent verticalstriations due to loss of secondary

    horizontal trabecula

    General osteoporosis. There is reducedradiographic density (osteopenia), with

    reduction in the number of trabeculae,which may be destroyed completely,and the bone cortex becomes thinned,as evident in the lateral radiograph ofthe calcaneus.

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    General osteoporosis. There is reducedradiographic density (osteopenia), withreduction in the number of trabeculae,which may be destroyed completely,

    and the bone cortex becomes thinned,as evident in the radiograph of the

    phalanx.

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    Sudecks Atrophy Demineralization of bone with intact butvery thin cortices.

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    Radiographic distinction of osteomalacia fromosteoporosis is often difficult.

    Frequently, the two conditions coexist.

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    Osteonecrosis is a disease of impairedosseous blood flow.

    Neither the etiology nor the natural historyhas been definitively determined.

    Many of the patients are young when they arediagnosed.

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    TRAUMATIC CONDITIONS :

    A displaced fracture of the femoral neck or a hip dislocation

    are wellknown etiologies of ONH; they lead to a mechanicalinterruption of the circulation to the femoral head.

    NONTRAUMATIC CONDITIONS :

    - Corticosteroid administration (respiratory and rheumatoddeseases, organ transplantation as well as Cushing disease)have a somewhat higher prevalence of osteonecrosis.

    - Excessive alcohol intake has been identified as an etiologicfactor.

    - Many other pathologies has been associated withOsteonecrosis : hemoglobinopathies, Dysbaric osteonecrosis,Gaucher desease, lupus, high-dose radiation, chemotherapy,

    hyperlipidemia...

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    The Xray findings : Early on are completely normal, but with ongoing

    problem, one sees progressively : Osteopenia Central radiolucency with a sclerotic border

    A Subchondral lucency - making up the crescentsign Fattening of the head. Collapse of the head. Secondary changes of Osteoarthritis. Joint space narrowing Progressive joint destruction

    Since the X-Rays may be completely normal - it is essentialto be able to see the problem early. Suggest an MRI ifhighly-suspected

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    These x-rays of the hip show thedifferent stages of the disease.

    - At first (stage I), there are nodetectable changes on x-ray (figA).

    - In stage II, there are some changesbut the surface is still intact (figB).

    -As the disease progresses, thesurace begins to collapse (fig C)until,

    -finally, the integrity of the joint isdestroyed (fig D).