Summary
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.
Keywords
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Article info
Publication history
Published online: July 08, 2013
Accepted:
May 15,
2013
Received:
December 17,
2012
Identification
Copyright
© 2013 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Inc. All rights reserved.
