Orbitotemporal neurofibromatosis: a case report

ABSTRACT

Neurofibromatosis is an autosomal dominant abnormality that may affect multiple organ systems. The eyelids, the orbits, the adjacent tissues and bones may be involved with varying frequency and severity. The management of orbitotemporal neurofibromatosis depends very much on the type and severity of the orbital involvement and on the functional state of the eye. Experience with surgical management of orbitotemporal neurofibromatosis involved in the orbit, the temporal soft tissue and bone with blind eye is reported. The goal of surgery is tumor resection, reconstruction of the orbital socket, aesthetic eyelids, and insertion of the artificial prosthesis. A two stage approach is recommended. In the first stage, tumor is resected and the orbital socket is reconstructed with titanium mesh plate and cranial bone graft. After reconstruction of the orbital socket, galeal flap is rotated posteriorly to cover the mesh plate and canthopexy is accomplished. Mask lift is performed to enhance aesthetics. In the second stage, correction of the bulky eyelids is achieved and orbital space for insertion of the artificial prosthesis is reconstructed. Authors have managed a orbitotemporal neurofibromatosis with blind eye of a 41-year-old male using titanium mesh plate and bone graft with satisfactory results.