Mod 4-1 Paget's Disease

Question 1

Who described all forms of Paget’s disease?

Answer 1

Sir James Paget

Question 2

Paget’s disease is the most common what?

Answer 2

Chronic metabolic disease

Question 3

What else has Paget’s disease been known as? Why?

Answer 3

Osteitis deforming 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.

Question 4

What happens in the early stages of Paget’s disease?

Answer 4

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

Question 5

What is the etiology of Paget’s disease?

Answer 5

Unknown but appears to have hereditary component as well as some link to a measles-related antigen.

Question 6

What are the two forms of Paget’s disease?

Answer 6

Monostotic (one bone) and Polyostotic (many bones)

Question 7

When Paget’s disease affects one bone (monostotic) what bone is most commonly affected?

Answer 7

The tibia

Question 8

What form of Paget’s disease is most common?

Answer 8

Polyostotic

Question 9

In polyostotic Paget’s disease, what sites re usually the first affected?

Answer 9

Pelvis and weight bearing bones of the lower extremity.

Question 10

List Paget’s disease site examples.

Answer 10

Pelvis, long bones of lower extremity, skull, vertebrae, clavicles and ribs.

Question 11

How does skeletal Paget’s disease progress?

Answer 11

In phases, most commonly 2 phase process.

Question 12

What occurs in Phase 1 of Paget’s disease?

Answer 12

• A large amount of blood is shunted to the affected bone and the bone becomes softened and volume is lost as bone mass is reabsorbed.
• Considered the destructive phase
• W/o the strengthening effect of osteoblastic activity, the bone(s) weaken and weight bearing structures may bow.

Question 13

What occurs in phase 2 of Paget’s disease?

Answer 13

• Repairative phase
• The bone becomes dense as sclerosis predominates, new bone is brittle with thickened/enlarged appearance, fractures may occur

Question 14

What do the symptoms of Paget’s disease depend on?

Answer 14

• Site of lesions
• Severity of lesions

Question 15

What percentage of patients with Paget’s disease have no symptoms?

Answer 15

20%

Question 16

Lesions that are located where seem to cause the most difficulty in Paget’s disease?

Answer 16

Long bones

Question 17

What can happen in an advanced case of Paget’s disease?

Answer 17

The weakened bone may fracture with even a slight blow or vertebral collapse.

Question 18

What is the most common presenting symptom of Paget’s disease?

Answer 18

Pain

Question 19

List clinical manifestations of Paget’s disease

Answer 19

• Pain (most common)
• Pathologic fractures
• Headache
• Tinnitus

Question 20

What is osteoporosis circumscripta?

Answer 20

refers to discrete radiolucent regions of the skull on plain radiographs

Question 21

What happens to the skull as sclerosis increases?

Answer 21

Enlarges

Question 22

As the cranium softens, expands and becomes heavier what else happens to the cervical area?

Answer 22

Bears down on cervical vertebrae, depressing the brainstem putting pressure on the cranial nerves leading to hearing loss and lightheadness and may eventaully lead to the collapse of C1 which leads to compression of the spinal cord and neural deficits.

Question 23

What fraction of paget’s disease cases affect the pelvis?

Answer 23

2/3

Question 24

What part of the pelvis is often affected?

Answer 24

The pubis and ischium and will show some degree of enlargement

Question 25

What does the book say about the pelvis in Paget’s disease?

Answer 25

It is often the initial site of Paget’s with the iliac rim of the pelvic brim (superior and medial to the acetabulum) to be the first bony areas affected.

Question 26

How does the destructive phase of Paget’s disease progress in the long bones?

Answer 26

Begins at one articular end and progresses towards teh other end creating a “blade of grass” appearance.

Question 27

When only one lesion is present with Paget’s disease, it’s commonly seen in the ______ area. What form of Paget’s disease is this?

Answer 27

Tibial; monostatic

Question 28

Describe phase 1 of Paget’s disease

Answer 28

• Areas of destruction (lucency)
• Lucency seen as osteoporosis circumscripta
• “Blade of grass” appearance in long bones

Question 29

Describe phase II of paget’s disease

Answer 29

• Areas of sclerosis (dense new bone)
• Thickening
• Repairative phase
• Cortex develops coarser, trabecular pattern
• Structure enlarges as new bone is laid down
• Patchy pattern of sclerosis appears when cranium is involved called “cotton wool” pattern

Question 30

What happens as bone weakens in Paget’s disease?

Answer 30

• Pathologic fractures
• Bowing (deformation)

Question 31

What is the effect on radiographic technique?

Answer 31

• Depends on phase of disease
• Osteoporosis is destructive
• Sclerosis is additive