Craniofacial fibrous dysplasia: A 10-case series

Eur Ann Otorhinolaryngol Head Neck Dis. 2017 Sep;134(4):229-235. doi: 10.1016/j.anorl.2017.02.004. Epub 2017 Mar 14.

Abstract

Objectives: 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.

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.

Keywords: Bisphosphonate; Bone tumor; Craniofacial fibrous dysplasia; Fibrous dysplasia; Optic neuropathy.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Bone Density Conservation Agents / administration & dosage*
  • Child
  • Diagnosis, Differential
  • Diphosphonates / administration & dosage*
  • Facial Bones / abnormalities*
  • Female
  • Fibrous Dysplasia, Polyostotic / diagnosis*
  • Fibrous Dysplasia, Polyostotic / therapy*
  • Follow-Up Studies
  • Humans
  • Male
  • Middle Aged
  • Pamidronate
  • Plastic Surgery Procedures / methods
  • Retrospective Studies
  • Skull / abnormalities*
  • Syndrome
  • Treatment Outcome

Substances

  • Bone Density Conservation Agents
  • Diphosphonates
  • Pamidronate