Skeletal disorders
The commonest skeletal disorder is dysostosis multiplex:
- bone formation is abnormal in the metaphysis, diaphysis and epiphysis
- radiographs demonstrate:
- oar shaped ribs - the flattened end at the sternum
- enlarged diaphyses and roughened epiphysial centres
- anterior beaking of the vertebrae
- odontoid hypoplasia
- rarely, spinal dislocation
Patients, notably with Gaucher's disease, may suffer vascular damage to bone such as avascular necrosis of the femoral head.
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