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PAGETโ€™S DISEASE
( Osteitis deformans)
July 2012 DR. FRANTZEN
DEFINITION
โ€ข Non-inflammatory condition of bone
โ€ข Furious bone resorption
โ€ข Chaotic bone deposition
Sir John Paget (1876)
July 2012 DR. FRANTZEN
INCIDENCE
โ€ข Some 3% of European population
( 90% asymptomatic )
โ€ข RSA : - 1.3% among black population
- 2.4% among white population
โ€ข Prevalence increases with age (> 40)
โ€ข Male:Female ratio = 3:2
July 2012 DR. FRANTZEN
Pathogenesis
Slow virus infection :
Paramyxovirus
July 2012 DR. FRANTZEN
CLINICAL FEATURES
โ€ข Initially asymptomatic
โ€ข Bone pain
โ€ข Stress fractures
โ€ข Increasing head size
โ€ข Blindness
โ€ข Deafness
โ€ข Bowing of legs
โ€ข Kyphosis
โ€ข High output cardiac failure
July 2012 DR. FRANTZEN
July 2012 DR. FRANTZEN
PATHOLOGY
โ€ข 3 PHASES
1. Osteolytic or hot phase
2. Mixed or intermediate phase
3. Sclerotic or cool phase
July 2012 DR. FRANTZEN
SKELETAL DISTRIBUTION
โ€ข Virtually any bone can be affected
Pelvis and tibia most common
Next femur, skull, spine and clavicle
July 2012 DR. FRANTZEN
CLINICAL
โ€ข Asymptomatic in 70%
โ€ข Bone pain
โ€ข Limb feels warm
โ€ข Deformities
โ€ข Neurological symptoms
โ€ข High output heart failure
July 2012 DR. FRANTZEN
COMPLICATIONS
Micro fractures
Bowing of femurs and tibias
Secondary OA of joints
Malignant transformation
Compression fractures of spine
High output heart failure
July 2012 DR. FRANTZEN
DIAGNOSIS
โ€ข Clinical picture
โ€ข Radiographs
โ€ข S-ALP , u-DPD, u-Hydroxyproline
โ€ข Bone scan
July 2012 DR. FRANTZEN
RADIOLOGY
โ€ข OSTEOLYTIC PHASE
Active bone resorption
Large areas of radiolucency
Destruction of cortex and
cancellous bone
July 2012 DR. FRANTZEN
RADIOLOGY
โ€ข INTERMEDIATE PHASE
Thickening of cortex and course
trabeculation of cancellous bone
Trabecular thickening along stress
lines
July 2012 DR. FRANTZEN
RADIOLOGY
โ€ข SCLEROTIC PHASE
Enlargement and widening of bone,
marked cortical thickening
with blurring of cortex-spongiosa
demarcation
July 2012 DR. FRANTZEN
TREATMENT
โ€ข Treatment does not cure Pagets,
but provides prolonged periods of
remission
July 2012 DR. FRANTZEN
TREATMENT
โ€ข Pain relief โ€“ NSAIDS
- Paracetamol
- Opioids
โ€ข Pharmacotherapy
- Biphosphonates
- Calcitonin
- Calcium 1000mg/d
- vit D
- exercise
July 2012 DR. FRANTZEN
TREATMENT
โ€ข Surgery indicated :
1. Unstable # and delayed union
2. OA for joint replacement
3. Osteotomy for deformities
4. Nerve compression in spine
July 2012 DR. FRANTZEN

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