title: McCune-Albright syndrome with craniofacial dysplasia: Clinical review and surgical management.
abstract: BACKGROUND
  Fibrous dysplasia (FD) is a benign fibro-osseous lesion related to an abnormal bone development and replacement by fibrous tissue. FD has three clinical patterns namely monostotic, polyostotic, and the McCune-Albright syndrome (MAS). MAS is a rare genetic disorder (about 3% of all FD's) that comprises a triad of polyostotic FD, café-au-lait skin macules, and precocious puberty. MAS can involve the orbit region and cause stenosis in the optic canal, leading the patient to a progressive visual loss.
  
  
  METHODS
  We reported a case of craniofacial FD in MAS in a 9-year-old male with progressive visual loss, submitted to optic nerve decompression by fronto-orbito-zygomatic approach, with total recovery. A research was made at Bireme, PubMed, Cochrane, LILACS, and MEDLINE with the keywords: FD/craniofacial/McCune-Albright/Optic compression for the clinical review.
  
  
  RESULTS
  A clinical review of the disease was made, the multiple, clinical, and surgical management options were presented, and the case report was reported.
  
  
  CONCLUSIONS
  MAS is a rare disease with a progressive polyostotic FD. Whenever it affects the orbit region, the optic canal, and it is associated with a progressive visual loss, the urgent optic nerve decompression is mandatory, either manually or with a rapid drill. It is known that aggressive approach is associated with less recurrence; it is also associated with worsening of the visual loss in optic nerve decompression. In MAS cases, multiple and less aggressive surgeries seem to be more suitable.
authors.affiliation: Department of Neurosurgery, Hospital e Maternidade Celso Pierro, PUC-Campinas, São Paulo, Brazil.
authors.forename: Telmo Augusto Barba
authors.initials: TA
authors.lastname: Belsuzarri
authors.affiliation: Department of Neurosurgery, Hospital e Maternidade Celso Pierro, PUC-Campinas, São Paulo, Brazil.
authors.forename: João Flavio Mattos
authors.initials: JF
authors.lastname: Araujo
authors.affiliation: Department of Neurosurgery, Hospital e Maternidade Celso Pierro, PUC-Campinas, São Paulo, Brazil.
authors.forename: Carlos Alberto Morassi
authors.initials: CA
authors.lastname: Melro
authors.affiliation: Department of Neurosurgery, Hospital e Maternidade Celso Pierro, PUC-Campinas, São Paulo, Brazil.
authors.forename: Maick Willen Fernandes
authors.initials: MW
authors.lastname: Neves
authors.affiliation: Department of Neurosurgery, Hospital e Maternidade Celso Pierro, PUC-Campinas, São Paulo, Brazil.
authors.forename: Juliano Nery
authors.initials: JN
authors.lastname: Navarro
authors.affiliation: Department of Neurosurgery, Hospital e Maternidade Celso Pierro, PUC-Campinas, São Paulo, Brazil.
authors.forename: Leandro Gomes
authors.initials: LG
authors.lastname: Brito
authors.affiliation: Department of Neurosurgery, Hospital e Maternidade Celso Pierro, PUC-Campinas, São Paulo, Brazil.
authors.forename: Luis Otavio Carneiro
authors.initials: LO
authors.lastname: Pontelli
authors.affiliation: Department of Neurosurgery, Hospital e Maternidade Celso Pierro, PUC-Campinas, São Paulo, Brazil.
authors.forename: Luis Gustavo
authors.initials: LG
authors.lastname: de Abreu Mattos
authors.affiliation: Department of Neurosurgery, Hospital e Maternidade Celso Pierro, PUC-Campinas, São Paulo, Brazil.
authors.forename: Tiago Fernandes
authors.initials: TF
authors.lastname: Gonçales
authors.affiliation: Department of Neurosurgery, Hospital e Maternidade Celso Pierro, PUC-Campinas, São Paulo, Brazil.
authors.forename: Wolnei Marques
authors.initials: WM
authors.lastname: Zeviani
keywords: Craniofacial dysplasia; McCune–Albright syndrome; fibrous dysplasia; orbit fibrous dysplasia; visual loss