Radical approach in the treatment of neurofibroma

The infiltrating character of plexiform and diffuse neurofibroma poses a difficult surgical challenge. The conventional approach of multiple partial resections of the tumor, and the soft character of the remaining tumor with additional changes in the surrounding tissue leave poor cosmetic results. In addition, the frequent rebound growth of the tumor after each partial resection creates a serious functional problem. The case presented here illustrates a classical clinical appearance and evolution of neurofibroma in the oculo-adnexal region in generalized neurofibromatosis (von Recklinghausen's disease). The conventional surgical approach consisting of repeated partial resections of the tumor resulted in a rebound growth to the stage of a total hemifacial disfigurement. The radical surgical approach applied then, and the subsequent plastic restorations, have not only eliminated the tumor but also restored the normal contour of the face. This case illustrates that the patient could have benefited from a radical approach in an earlier stage of the disease.