RESUMO
OBJECT: Plexiform neurofibroma is a characteristic lesion of Von Recklinghausen's disease. Conservative surgery is the most widely adopted treatment. However, it is very challenging because of its hemorrhagic nature and the infiltrative aspect of the lesions. The aim of this study was to evaluate our management. PATIENTS AND METHOD: A retrospective study over 16 years was realized and during this period 35 patients with neurofibroma with cervico-facial location were included. RESULTS: There were 18 men and 17 women with an average age of 23 years (3-50 years). The familial form was found in 9% of patients. Aesthetic discomfort was noted in all patients and functional impairment only occurred in 10% of patients. The NFP was localized at the hemiface in 11 cases, periorbital in 6 cases, naso-labial in 5 cases, scalp in 4 cases, jugal in 4 cases and cervico-chin in 5 cases. Size of the lesions averaged 11.6cm (4-45cm). Eighteen patients (51.4%) were operated including 10 by modeling resection, 05 cervico-facial lifting and 3 complete resections. Complication rate was 28% dominated by disunion wound. The average number of procedures was 1.6 (1 to 5). After 3 years average follow-up, aesthetic et functional results was assessed as good over 75 per cent of patients. CONCLUSION: Cervico-facial plexiform neurofibromas is challenging. Conservative surgery should be the gold standard and long time follow-up is recommended.
