Pathology pagets disease

destructive osteoporosis

- technique

phase 1

Destructive hyperemia bowing may occur

one of the most common chronic metabolic diseases of the skeleton

Paget's disease

___% of the diagnosed cases there are no symptoms at all

20

commonly affected and will show some degree of enlargement as they become involved

The pubis and the ischium

phase 1 is destructive because:

Without the strengthening effect of accompanying osteoblastic activity, the bone weakens, and if it is a weight bearing structure, bowing may occur as it attempts to support the patient's weight.

polyostotic

affect many bones most common

Early stage of disease

bone is rapidly resorbed (seen as areas of lucency) and replaced with bone of a coarse, irregular consistency (seen as areas of sclerosis) that becomes weak and fractures easily.

Paget's disease

bony disease characterized by distortion of bony architecture as a result of increased turnover of osteoclasts and osteoblasts

sclerosing phase

cotton wool appearance As the sclerosing increases, the size of the skull actually enlarges.

destructive phase radiographically

destroyed the outer table of the skull in the frontal and inferior areas

As the sclerotic phase progresses, the bone becomes

enlarged and thickened, as mentioned extremely weak and subject to easy fracture

Risk factors

familial link infective component

As the cranium softens, expands and becomes heavier in the sclerosing phase what happens

it bears down on often involved upper cervical vertebrae

Paget's disease is also known as

osteitis deformans because the disease is characterized by localized widespread distortion or deformity of the architecture of affected bone(s) caused by an increased remodeling of bone by osteoblasts and osteoclasts

destructive pattern is so characteristic of Paget's that it has it's own name

osteoporosis circumscripta

most common symptom

pain

Clinical manifestatons

pain pathologic fractures headache tinnitus

what is affected in 2/3rds of paget cases

pelvis As the disease progresses, there is the familiar pattern of trabecular coarsening

Areas of destruction (lucency) are part of

phase I seen as osteoporosis circumscripta in the skull and with a "blade of grass" appearance in extremities

Phase 2

reparative sclerosis and cortical thickening brittle, prone to fracture

osteoporosis circumscripta has what kind of outlined area

sharply outlined area of lucency is clearly visible.

monostotic

that it affects a single bone, most commonly the tibia

phase 2 the reparative phase what happens to the bone

the bone becomes increasingly dense as sclerosis predominates, but the new bone is brittle in spite of its thickened and enlarged appearance

In phase 1, a large amount of blood is shunted to the affected bone

the bone becomes softened and volume is lost as bone mass is reabsorbed

The disease disturbs the growth of new bone tissue with the result of:

the bones often thicken, become soft, and coarsen in texture.

as it bears down involving the c- vertebrae what happens to the brainstem

the brainstem becomes depressed, putting pressure upon a variety of cranial nerves that lead to, among other things, hearing loss and lightheadness

that the pelvis is often the initial site of Paget's, with :

the iliac rim of the pelvic brim, that portion of the ilium superior and medial to the acetabulum, considered to be one of the first bony areas affected

polyostotic most common affected bone

the pelvis and the weight bearing bones of the lower extremity are usually the first sites affected

Areas of sclerosis (dense new bone) and thickening represent phase II

the reparative phase of the disease. The cortex of the affected bone develops a coarser trabecular pattern, the involved structure enlarges as new bone is laid down, a patchy pattern of sclerosis appears when the cranium is affected, called the "cotton wool" pattern.

Symptoms of Paget disease of the bone depends on

the site of the lesions and the severity of the lesions

Ilium becomes

thickened, sclerotic and coarse

When only one lesion is present with Paget's, it commonly is seen in

tibial area

In an advanced case, the weakened bone may fracture

with even a slight blow, or, as in the case of the vertebrae, may collapse

Etiology

not always clear. appears to have a hereditary component -as well as some link to a measles-related antigen

In the long bones, the destructive phase disease always begins at

one articular end and progresses towards the other end, sometimes creating a "blade of grass" appearance

sclerosis technique

+

cause the most difficulty

Lesions in the long bones

As a result of the brittleness

fractures may occur, with the bones snapping in half like a piece of chalk.

Examples/sites

monostotic or polyostotic