Surgical Management of Diffuse Plexiform Neurofibroma in Von's Disease Recklinghausen: About a Case at Mopti Hospital

Abstract

Introduction: Facial plexiform neurofibromas are considered a rare but disfiguring and devastating complication of facial neurofibromatosis. The objective of this work is to show the difficulties of the surgical management of the facial manifestations of neurofibromatosis. Observation: A 39-year-old woman consulted for a monstrous-looking swelling with an irregular texture on the left hemiface, evolving for 13 years. She underwent external carotid artery ligation, total tumor excision and thin skin grafting. Histology concluded to a facial plexiform neurofibroma. Discussion: Neurofibroma is a benign tumor arising from the connective elements of the Schwann sheath by proliferation of the endoneurial matrix. Complete surgical excision is the treatment of choice for neurofibroma. Conclusion: Facial neurofibromatosis surgery remains a challenge for the surgeon. We must have a critical look at our gestures and indications. In particular, provide for surgery after the age of puberty, ligation of the external carotid artery and reconstructive cosmetic surgery.