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