Pelvic bones are sclerotic but with normal contours. Cortices of the long bones are sclerosed and hyperostotic, and their shafts undermodeled. Surgery to relieve intracranial pressure may be helpful. These symptoms usually resolve by age 30. Cranial nerve compression and raised intracranial pressure are occasional complications.
Some patients are severely handicapped, whereas others are virtually asymptomatic.
The predominant x-ray feature is marked thickening of the periosteal and medullary surfaces of the long bones' diaphyseal cortices. The medullary canals and external bone contours are irregular. The extremities and the axial skeleton usually are spared. Rarely, the skull is involved, with calvarial widening and basal sclerosis. X-ray changes are variable.