Paget's disease is usually asymptomatic and is often discovered incidentally on x-ray or during the investigation of an unrelated disorder (1).
The presentation of symptomatic disease is extremely variable and depends upon the extent of the disease and associated complications. The sites most commonly affected are the pelvis (71%), lumbar spine (51%), femur (49%) and thoracic spine (43%). This disease rarely affects the appendicular bones e.g. bones of the hand and feet (1).
- pain
- bone pain and joint pain are common presenting complaint
- results from a combination of microfractures, compression of nerves as they pass through bony foramina and increased vascularity or coexisting arthritis.
- characteristically begins late in the clinical course,
- occurs throughout the day and at rest and often worse at night
- pain in femur or tibia often increases on weight bearing (especially in the presence of osteolytic lesions) (1)
- bone deformities
- common
- weakness may cause the long bones to bend under stress
- anterior bowing of the tibia and femur occur frequently
- overlying skin is often unduly warm
- considerable kyphosis is not uncommon and the patient may assume an ape-like posture with bent legs and arms hanging in front.
- fractures
- thickening of the calvarium may occur with the patient complaining that hats no longer fit.
- may also be associated with visual loss, facial palsy and trigeminal neuralgia.
- shortening of the skull base or platybasia, may create the impression of a shorter neck. More seriously, it may cause narrowing of the foramen magnum and threaten the medulla.
- hearing loss - due to impingement on the eighth cranial nerve. Deafness may also be the result of Pagetic change in the ossicles of the ear.
- thickening of the vertebra may cause compression of the nerve roots with pain felt in the back and the lower.
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