A 58-year-old woman noticed that her front teeth had become spaced and seeks advice from her dentist. On entering the surgery, the dentist notices that she has difficulty in walking and does not respond to his questions. She has become increasingly deaf and her vision has also deteriorated. On examination, the maxilla and zygoma are enlarged and there is enlargement of the forehead.
1. What diagnosis would you suspect?
Pagetʼs disease of bone results in enlargement of cranial bones and deformation of weight- bearing bones. The cranium is usually expanded in thickness and symptoms may arise from cranial nerve compression.
2. What information might be gained from oral radiographs and blood tests to support this diagnosis?
Radiographs of the jaws may show hypercementosis, cemental masses, abnormal trabeculation and a cotton-wool appearance in the jaws. The alkaline phosphatase level is markedly raised.
3. What are the principal histological features of this disorder?
Disordered bone remodelling is seen; larger osteoclasts are present and the trabeculae show a scalloped outline. Numerous resting and reversal lines, resulting in a mosaic pattern, are seen and the vasculature may be increased. Globular cementum-like masses are seen in the jaws.