Resumo

LANDRIEL, Federico; FERRARA, Paula; HEM, Santiago  e  CARRIZO, Antonio. Neurofibromatosis segmentaria fronto témporo orbitaria: Reporte de un caso. Revisión de la literatura. Rev. argent. neurocir. [online]. 2007, vol.21, n.3. ISSN 1850-1532.

Objective. To report a case of segmental neurofibromatosis located in the fronto temporal orbitary region. Description. A 54-year-old patient with a right progressive exoftalmos due to a tumor inside the orbit. He was operated 18 years ago with an histopatology diagnosis of neurofibroma. He recovered well from the surgery, but 3 years ago the right exoftalmos reappeared. The MRI shows multiple subgaleal tumors located in the right fronto temporal area, inside and outside the orbit. Intervention. A right fronto orbitary craniotomy was performed with the resection of soft non encapsulated subgaleal lesions. Conclusion. Segmental neurofibromatosis is a rare afection wich is very difficult to deal with, because of the high incidence of recurrence of the neurofibromas. Despite this, in cases like this one a surgical treatment is highly recommended to prevent not only visual defects but also cosmetic alterations.

Palavras-chave : Neurofibromatosis of face; Neurofibromatosis type 5; Orbitary neurofibromatosis; Segmental neurofibromatosis.

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