Epidemiology
A fibro-osseous lesion linked to a postzygotic mutation, which can affect one bone or several bones. New pain in a stable lesion can indicate a fracture, progressive deformity, or another complication that warrants reassessment.
Basic features
It can cause pain, deformity, or fracture, or be an incidental finding, with a ground-glass pattern; in its polyostotic form it may be associated with McCune-Albright syndrome (café-au-lait spots and endocrine abnormalities).
Usual treatment
It is managed with pain relief and, for persistent bone pain, intravenous bisphosphonates (such as pamidronate or zoledronate), which relieve pain but do not change the size or the course of the lesion; it also involves endocrine control when appropriate. When surgery is needed, the preference is stabilization with load-sharing implants (intramedullary nails) and corrective osteotomies: curettage with bone grafting is not indicated, because the graft is resorbed and the lesion returns.
Outcomes and prognosis
It usually stabilizes after skeletal maturity, but extensive lesions can leave mechanical sequelae and require long-term follow-up.