Craniofacial fibrous dysplasia: A 10-case series
Résumé
Objective: Fibrous dysplasia of bone is a rare sporadic benign congenital condition in which normal cancellous bone is replaced by fibro-osseous tissue with immature osteogenesis. Sarcomatous transformation is exceptional. Lesions may involve one bone (monostotic) or several (polyostotic). Fibrous dysplasia may be associated with cafe-au-lait skin macules and endocrinopathy in McCune-Albright syndrome, or with myxoma in Mazabraud's syndrome. Methods: We report ten cases of patients followed up for craniofacial fibrous dysplasia in our center between 2010 and 2015. Results: Mean age was 43 years (range, 10-72 years). Clinical symptoms comprised headache (n = 3) and sensorineural disorder: recurrent anterior uveitis (n = 1), visual acuity loss, epiphora and vestibular syndrome (n = 1), and hearing loss (n = 1). All cases were monostotic. The sphenoid bone was most commonly involved (n = 5), followed by the ethmoid (n = 1), frontal (n = 1), fronto-ethmoid (n = 1), temporal (n = 1) and fronto-ethmoido-sphenoid (n = 1) bones. Five patients were treated with intravenous pamidronate, a bisphosphonate: evolution was favorable for 3 of them at 1-6 months after treatment initiation, with resolution of headache or vestibular syndrome; the other 2 patients were stable. Two patients were operated on. Conclusion: Diagnosis of craniofacial fibrous dysplasia should be considered in case of headache, neuralgia, sensory disorder, functional disorder or infectious ENT complications. A medico-surgical approach is useful for these patients.