Neurofibromatosis segmentaria fronto temporo orbitaria: reporte de un caso: Revisión de la literatura / Fronto temporo orbitary segmental neurofibromatosis: Report of one case

ABSTRACT

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 affection 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.