Paediatric craniofacial fibrous dysplasia: the Hospital for Sick Children experience and treatment philosophy

Craniofacial fibrous dysplasia is a benign developmental anomaly in which normal bone is replaced by fibro-osseous tissue. The aim of this study was to audit the patient population at a tertiary paediatric centre and report our treatment protocols. A retrospective chart review of all patients with craniofacial fibrous dysplasia treated at the Hospital for Sick Children between 1999 and 2010 was performed. The treatment algorithm used by our centre is presented. A total of 55 patient records were reviewed; 37 patients had sufficient documentation for study; 27 (16 male, 11 female) patients underwent surgery at our institution, of these patients, 26 had post-operative follow up of greater than one year (mean 41 months; median 24 months). Mean age at presentation was 9.9 years (median 10 years) and mean age of surgery was 13 years. Ten patients underwent surgery on the fronto-orbital region, 7 of the calvarium, 2 the skull base and 8 upon tooth-bearing bones. Fourteen cases underwent debulking surgery as their primary therapy whereas 13 patients had complete resection. Nine patients experienced recurrence and all but one case of these occurred in patients that underwent debulking therapy. When age of surgery is considered, total resection and reconstruction or debulking surgery after skeletal maturity has a lower recurrence rate (1/7 cases) than earlier surgery (8/16). Complete resection at any age and debulking surgery once skeletal maturity has been reached may be associated with lower recurrence rates than incomplete resections at an earlier age. Patients with McCune-Albright syndrome may benefit from repeated debulking procedures rather than complex resections and reconstructions.