Background
Fibrous dysplasia of the anterior skull-base can lead to severe facial disfigurement with optic nerve compression and blindness. The management in children is controversial.

Objectives and Methods
We evaluate presentation and outcomes in our case series of paediatric anterior skull-base fibrous dysplasia from 2011 to date in a single UK tertiary centre with a review of the available literature.

Results
7 patients were managed presenting with a mean age of 7 years. 5 children had complete unilateral visual loss at presentation. 2 children required urgent optic canal decompression for sudden visual deterioration and both had objective improvement postoperatively. All children were commenced on Bisphosphonate therapy. No child had visual deterioration once medical therapy commenced and surveillance imaging remained stable.

Conclusions
Management dilemmas remain but bisphosphonate therapy with surgery to decompress the remaining optic canal reserved for objective evidence of acute deterioration seems to have been an effective strategy in our patients.

Management of anterior skull-base fibrous dysplasia in children