Paget’s Disease (aka Osteitis Deformans)

-Is a bone disease of unknown origin characterized by osteolysis followed by extensive attempts at repair.

1. Mimics: Paget’s disease mimics a Hemangioma. Hemangioma for the vertebral bodies produces a vertically striated pattern, which may closely mimic the vertical trabeculae of Paget’s disease.

2. Most likely site: Pelvis

Least likely site is the Fibula

3. List the 4 phases and describe each

   1. Stage one: Osteolytic, Destructive, or Monophasic stage- Osteoclastic over activity creates gross loss of bone density described as osteoporosis circumscripta

   2. Stage two: Combined, Mixed, or Biphasic stage – M/c encountered. Reflection of both destruction (Lytic) and production of bone (Blastic). Characterized by cortical thickening, increased radiopacity and accentuation of trabecular patterns with lucent areas mixed.

   3. Stage three: Sclerotic or Ivory Stage – Uniform thickening of trabeculae with ivory appearance

   4. Stage Four: Malignant Degeneration – Lethal stage

4. Clinical Features of Paget’s

   1. 2:1 males, m/c after 55

   2. 90% are asymptomatic. Pain, when present, is low intensity and may be associated with bowing deformities or fractures

   3. Increased hat size because enlargement of the calvaria

5. Radiological features

   1. Bone scan will be hot

   2. Skull will demonstrate in earl lesions Osteoporosis circumscripta (described in c). More advanced or combined stage demonstrates cotton wool appearance, which is fuzzy, poorly defined edges of sclerotic areas.

   3. Spine will demonstrate squared-off picture frame vertebra, which is thickened and enlarged vertebral endplates giving a squared look.

   4. Homogenous increases radiopacity of vertebral body and creates an ivory vertebra

      1. The 3 m/c causes of an ivory vertebra are: osteoblastic metastatic carcinoma, Paget’s disease, and Hodgkins lmphoma

Category: Orthopedics Notes