title: Craniofacial Fibrous Dysplasia Involving the Orbit: A Case Report and Literature Review.
abstract: OBJECTIVE
  Craniofacial fibrous dysplasia is a benign slowly progressive disease in which normal craniofacial bones are replaced by immature fibro-osseous tissue. Orbital involvement may present as visual loss in some patients. The etiology of visual loss in orbital fibrous dysplasia is still unclear, with many theories proposed in recent literature. This has led to developments in the controversy regarding the role of prophylactic optic nerve decompression in asymptomatic orbital fibrous dysplasia patients. This case report and literature review evaluates the need for early surgical intervention in such patients, as well as highlights other aspects of the condition and its management.
  
  
  METHODS
  Case report and literature review.
  
  
  METHODS
  This was a case report of a 12-year-old Chinese girl who developed progressive visual loss in association with craniofacial fibrous dysplasia involving the orbit. Literature regarding the epidemiology, pathophysiology, clinical features, diagnosis, investigations, and management was searched from the PubMed database.
  
  
  RESULTS
  Extradural decompression of the optic nerve resulted in marked visual improvement, with temporal optic atrophy noted postoperatively. Literature review revealed a number of recent studies recommending against prophylactic optic nerve decompression, but early surgical intervention upon onset of visual loss is suggested.
  
  
  CONCLUSIONS
  Immediate surgical referral and early optic nerve decompression is recommended upon onset of visual loss. Periodic follow-up is recommended to monitor for recurrence and malignant transformation.
authors.affiliation: From *Yong Loo Lin School of Medicine, National University of Singapore, and †Ophthalmology and Visual Sciences, Khoo Teck Puat Hospital, Singapore.
authors.forename: Leonard Tang Yew
authors.initials: LT
authors.lastname: Leong
authors.forename: Benjamin Julian Chang Chong
authors.initials: BJ
authors.lastname: Ming
mesh_terms: D002648:Child; D019299:Decompression, Surgical / Q000379:methods*; D005147:Facial Bones; D005260:Female; D005359:Fibrous Dysplasia, Polyostotic / Q000175:diagnosis* / Q000601:surgery; D006801:Humans; D009915:Orbit; D012886:Skull; D014057:Tomography, X-Ray Computed