Surgical management of craniofacial neurofibromatosis type 1 associated tumors.

ABSTRACT

Neurofibromatosis type 1 is a rare, autosomal dominant disorder than can present with varying degrees of disfigurement depending on the associated tumor extent and location. Surgical resection is considered the most effective management of these typically benign tumors, indicated when symptoms include pain, extreme deformity, or interference with normal physical function. Giant tumors of the craniofacial region present particular difficulty due to the size of the post-resection wound deficit and the high risk surgery poses to function such as vision and facial animation in this region. Strategies of management are discussed.