{"id":"f4f8d306-fad8-4c7b-a77d-7a936b75dae9","slug":"mccune-albright-syndrome-with-craniofacial-dysplasia-clinical-review-and-surgical-management","title":"McCune–Albright syndrome with craniofacial dysplasia: Clinical review and surgical management","authors":["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"],"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.","thumbnailUrl":"https://sni-digital-videos.s3.amazonaws.com/articles/f4f8d306-fad8-4c7b-a77d-7a936b75dae9/featured/hero-1782420937009.png","publishDate":"2016-03-11T00:00:00.000Z","doi":"10.4103/2152-7806.178567","categories":["Pediatric Neurosurgery","Review Article"],"fullTextUrl":"http://surgicalneurologyint.com/wp-content/uploads/2016/03/6636/SNI-7-165.pdf"}