“ Also known as ‘osteitis deformans’, this is a common disorder of osteoclasts and osteoblasts that results in disordered and excessive bone...
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Also known as ‘osteitis deformans’, this is a common disorder of osteoclasts and osteoblasts that results in disordered and excessive bone remodelling. Bone marrow is replaced by fibrous tissue with vascular channels. A total of 10% of Paget disease cases are seen in those aged >80 years. The spine and pelvis (75%) and skull (up to 65%) are most commonly affected.
The stages of disease are the active osteolysis (osteoclasts dominate), mixed active phase (osteoclastic and blastic action) and late inactive phase (osteoblasts dominate).
PLAIN FILM
- Typically polyostotic and asymmetrical pattern.
- Flame/grass-shaped metadiaphyseal lucencies in active phase—large lucencies may appear in the skull, known as ‘osteoporosis circumscripta’.
- Key features of inactive disease are bone expansion, thickening of the cortex and coarsening of the trabecular pattern.
- Cotton wool sclerosis in the skull
SPINE X-RAY
• Picture frame appearance (due to cortical thickening)• Ivory vertebra• Coarsening of the vertical trabeculations
BONE SCAN
- 90% sensitive.
- Typically, there is markedly increased uptake in the bone lesion in all phases of disease, particularly during active disease.
- Uptake at the margins is seen with osteoporosis circumscripta.
- Decreased uptake of technetium-99m sulphur colloid by bone marrow.
MRI
- Appearance varies according to disease phase.
- Chronic Pagetic bone has similar signal characteristics to fat
Paget disease. Lateral tibia x-ray demonstrating a sabre tibia with cortical thickening, coarsening of the trabeculae and bowing of the tibia.
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