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Belsuzarri TA, Araujo JF, Melro CA, et al. McCune-Albright syndrome with craniofacial dysplasia: Clinical review and surgical management. Surg Neurol Int. 2016;7:S165-9doi: 10.4103/2152-7806.178567.
Belsuzarri, T. A., Araujo, J. F., Melro, C. A., Neves, M. W., Navarro, J. N., Brito, L. G., Pontelli, L. O., de Abreu Mattos, L. G., Gonçales, T. F., & Zeviani, W. M. (2016). McCune-Albright syndrome with craniofacial dysplasia: Clinical review and surgical management. Surgical neurology international, 7S165-9. https://doi.org/10.4103/2152-7806.178567
Belsuzarri, Telmo Augusto Barba, et al. "McCune-Albright syndrome with craniofacial dysplasia: Clinical review and surgical management." Surgical neurology international vol. 7 (2016): S165-9. doi: https://doi.org/10.4103/2152-7806.178567
Belsuzarri TA, Araujo JF, Melro CA, Neves MW, Navarro JN, Brito LG, Pontelli LO, de Abreu Mattos LG, Gonçales TF, Zeviani WM. McCune-Albright syndrome with craniofacial dysplasia: Clinical review and surgical management. Surg Neurol Int. 2016 Mar 11;7:S165-9. doi: 10.4103/2152-7806.178567. eCollection 2016. PMID: 27057395; PMCID: PMC4804399.
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Surg Neurol Int. 2016 Mar 11;7:S165-9. doi: 10.4103/2152-7806.178567. eCollection 2016.

McCune-Albright syndrome with craniofacial dysplasia: Clinical review and surgical management.

Surgical neurology international

Telmo Augusto Barba Belsuzarri, João Flavio Mattos Araujo, Carlos Alberto Morassi Melro, Maick Willen Fernandes Neves, Juliano Nery Navarro, Leandro Gomes Brito, Luis Otavio Carneiro Pontelli, Luis Gustavo de Abreu Mattos, Tiago Fernandes Gonçales, Wolnei Marques Zeviani

Affiliations

  1. Department of Neurosurgery, Hospital e Maternidade Celso Pierro, PUC-Campinas, São Paulo, Brazil.

PMID: 27057395 PMCID: PMC4804399 DOI: 10.4103/2152-7806.178567

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.

CONCLUSION: 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.

Keywords: Craniofacial dysplasia; McCune–Albright syndrome; fibrous dysplasia; orbit fibrous dysplasia; visual loss

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