Recommended management for fibrous dysplasia in individuals with fibrous dysplasia / McCune-Albright syndrome

b.i.d. = twice daily; CT = computerized tomography; FD = fibrous dysplasia; GI = gastrointestinal; NSAIDs = nonsteroidal anti-inflammatory drugs

1. Affected individuals should be evaluated yearly by a neuro-ophthalmologist; less frequently once stability is demonstrated. Those with evidence of optic neuropathy should be referred to an experienced craniofacial surgical team.

2. Repeat head CT approximately every five years, potentially sooner in younger individuals, those with severe disease, or if vision or hearing deficits develop [Boyce et al 2017].

3. Optic nerve encasement is common and usually asymptomatic. Prophylactic optic nerve decompression in the absence of optic neuropathy is contraindicated [Lee et al 2002, Amit et al 2011].

4. Scoliosis may be progressive and potentially fatal in severe cases. All affected individuals with scoliosis should be followed regularly by an orthopedic surgeon [Leet et al 2004b].

5. Inadequately treated hypophosphatemia may significantly worsen bone pain and must be addressed before considering bisphosphonates [Leet et al 2004a, Paul et al 2014].

6. Bisphosphonates have not been shown to affect disease progression and use should be limited to treatment of FD-related bone pain [Hart et al 2007, Boyce et al 2014].

7. Doses should be repeated as needed when pain returns rather than on a set dosing schedule. In absence of significant decrease in bone pain, bisphosphonate treatment should be discontinued.