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Paget’s Disease of Bone

Title               

Ñ    Paget’s Disease of Bone

Definition

Ñ    Progressive focal disorder of bone remodelling

Ñ    Normal bone is removed and replaced by abnormal bone

Pathogenesis

Causes

Ñ    Unknown

Ñ    Gene predisposing to Paget’s identified on chromosome 18 q

Ñ    Paramyxovirus infection may be implicated

Pathological Process

Ñ    Increased number of osteoclasts which contain an increased number of nuclei

Ñ    Increased osteoclastic reabsorption of bone

Ñ    Replaced by woven bone

Ñ    This is softer

Ñ    Causes deformity

Ñ    Increased risk of fracture

Clinical Features

History

Ñ    Largely asymptomatic

Age

Ñ    Over 40 years

Sex

Ñ    Males: Females=2:3

LMS

Ñ    Bone pain

Ñ    Deformity

Ñ    Arthritis

Areas involved commonly (order of frequency):

Ñ    Pelvis, lumbar spine, femur, thoracic spine, sacrum, skull, tibia

Examination

CVS

Ñ    Increased blood flow causing localised warmth

LMS

Ñ    Deformity:

Bowed tibia, skull changes:

Common

Ñ    Osteoarthritis:

In adjacent joints

Complications

E&M

Ñ    Hypercalcaemia:

If immobilised

Ñ    Increased incidence of hyperparathyroidism:

Real or due to increased investigation in these patients

CVS

Ñ    Heart failure:

High output

CNS

Ñ    Angioid streaks in retina

Ñ    Hydrocephalus

Ñ    Deafness

Ñ    Platybasia

Ñ    Basilar invagination

Ñ    Spinal cord compression

LMS

Ñ    Pathological fracture

Ñ    Neoplastic change:

Osteosarcoma

Investigations

Fluids

Blood

Markers of bone formation:

Ñ    Alkaline phosphatase:

Increased

Urine

Markers of bone resorption:

Ñ    Urinary hydroxyproline:

Increased

Imaging

X-ray

Ñ    Lytic phase

Ñ    Mixed phase

Ñ    Sclerotic phase

Nuclear Medicine

⁹⁹ Tc labelled bisphosphonate

Ñ    Determine extent:

Hot spots

Management

Control

Drugs

Ñ    Bisphosphonates

Surgery

Ñ    Joint replacement:

For secondary osteoarthrosis

Ñ    Osteotomy:

For secondary osteoarthrosis

 

Ñ    Amputation:

Osteosarcoma

Ñ    Neurosurgery:

Spinal disease

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