RESUMO
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.
RESUMO
Angiolopomas da coluna vertebral constituem neoplasias raras (40 casos descritos na literatura). Em sua maioria estes tumores säo epidurais, apresentam características benignas, contendo elementos vasculares e elementos adiposos maduros. A sintomatologia é inespecífica, mas a tomografia computadorizada e principalmente a ressonância eletromagnética permitem fazer o diagnóstico com precisäo, como exemplifica o caso relatado
