Place of surgery in the treatment of plexiform nevroma of the face plexiform nevroma of the face: about 09 cases of von recklinghausen disease

Abstract

Neurofibromatosis 1 (NF1) is the most common neurofibromatosis. It’s a genetic disorder defined by the development of heterogeneous nerve tumors, neurofibromas. 40% of NF1 are revealed by a facial damage. The aim of our work is to present the current knowledge of NF1 and report, from the analysis of clinical cases, surgical indications of plexiform neurofibroma. This is a retrospective study conducted at the department of maxillofacial and aesthetic surgery CHU Mohammed VI Marrakech. Our study included 9 patients with plexiform neurofibroma of the face. The average age was 26.5 years (15 to 36 years). The sex ratio M / F was 1.The neurofibroma was unilateral in all patients. Dysplasia of the sphenoid greater wing was found in 2 patients, causing in a patient a cranial orbit, meningo-encephalocele and pulsatile exophthalmos. 6 patients were operated. And 2 refused. Surgery was iterative with an average of two interventions, and it was to reduce the tumor volume. The aesthetic result was satisfactory and allowed a rehabilitation of patients. There was a recurrence in a patient with a decline of 3 years. Surgery has an important place in the treatment of plexiform neuroma of the face. Its purpose is twofold, aesthetic and functional. It is only palliative because it does not change the evolution of the disease. Two problems arise during surgery: hemorrhagic and invasive nature of these tumors. The resections are often incomplete and intra-tumoral. There is no standardization of the timing and importance of surgery to effector especially in children. The degeneration of neurofibroma in neurofibrosarcoma is a rare but dangerous