title: Craniofacial fibrous dysplasia: A 10-case series.
abstract: 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 café-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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: CHU Clermont-Ferrand, Service de Médecine Interne, Hôpital Gabriel Montpied, 58, Rue Montalembert, 63003 Clermont-Ferrand, France.
authors.forename: A
authors.initials: A
authors.lastname: Couturier
authors.affiliation: Université Clermont Auvergne, CHU Clermont-Ferrand, Service de Médecine Interne, Hôpital Gabriel Montpied, Inserm U1071, INRA USC2018, M2iSH, 63000 Clermont-Ferrand, France.
authors.forename: O
authors.initials: O
authors.lastname: Aumaître
authors.affiliation: Université Clermont Auvergne, CHU Clermont-Ferrand, Service d'ORL, Hôpital Gabriel Montpied, UMR Inserm 1107 - Equipe Biophysique Neurosensorielle, 63000 Clermont-Ferrand, France.
authors.forename: L
authors.initials: L
authors.lastname: Gilain
authors.affiliation: CHU Clermont-Ferrand, Service de Radiologie, Hôpital Gabriel Montpied, 58, Rue Montalembert, 63003 Clermont-Ferrand, France.
authors.forename: B
authors.initials: B
authors.lastname: Jean
authors.affiliation: Université Clermont Auvergne, CHU Clermont-Ferrand, Service d'ORL, Hôpital Gabriel Montpied, UMR Inserm 1107 - Equipe Biophysique Neurosensorielle, 63000 Clermont-Ferrand, France.
authors.forename: T
authors.initials: T
authors.lastname: Mom
authors.affiliation: Université Clermont Auvergne, CHU Clermont-Ferrand, Service de Médecine Interne, Hôpital Gabriel Montpied, Inserm U1071, INRA USC2018, M2iSH, 63000 Clermont-Ferrand, France. Electronic address: mandre@chu-clermontferrand.fr.
authors.forename: M
authors.initials: M
authors.lastname: André
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D050071:Bone Density Conservation Agents / Q000008:administration & dosage*; D002648:Child; D003937:Diagnosis, Differential; D004164:Diphosphonates / Q000008:administration & dosage*; D005147:Facial Bones / Q000002:abnormalities*; D005260:Female; D005359:Fibrous Dysplasia, Polyostotic / Q000175:diagnosis* / Q000628:therapy*; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D000077268:Pamidronate; D019651:Plastic Surgery Procedures / Q000379:methods; D012189:Retrospective Studies; D012886:Skull / Q000002:abnormalities*; D013577:Syndrome; D016896:Treatment Outcome
chemical_list: D050071:Bone Density Conservation Agents; D004164:Diphosphonates; D000077268:Pamidronate
keywords: Bisphosphonate; Bone tumor; Craniofacial fibrous dysplasia; Fibrous dysplasia; Optic neuropathy