[Difficult surgical management of facial neurofibromatosis type I or von Recklinghausen disease in children].
We report a case of predominantly facial neurofibromatosis type I in a 7-year-old girl, exposing the difficulties encountered in surgical management. Infiltration of facial soft tissues by plexiform neurofibroma is difficult to control. Resection of large areas of facial skin is unconceivable, facial functions must be preserved. Orbitocranial surgery is reserved for orbital manifestations of neurofibromatosis with osseous sphenoid dysplasia and pulsatile exophthalmos. Despite progress in tumor imaging and surgery, facial neurofibromatosis remains a challenge for the surgeon. We discuss surgical technique and indications for surgery in these cases.
Heuze
Y
Y
Clinique de Stomatologie et Chirurgie Maxillo-Faciale, CHU Hôtel-Dieu, 1, Place Alexis Ricordeau, 44093 Nantes Cedex 01, France.
Piot
B
B
Mercier
J
J
D002648:Child; D002675:Child, Preschool; D005094:Exophthalmos / Q000601:surgery; D005153:Facial Neoplasms / Q000473:pathology / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D009361:Neoplasm Invasiveness; D018318:Neurofibroma, Plexiform / Q000473:pathology / Q000601:surgery; D009456:Neurofibromatosis 1 / Q000473:pathology / Q000601:surgery*; D009918:Orbital Neoplasms / Q000601:surgery; D012878:Skin Neoplasms / Q000473:pathology / Q000601:surgery; D012888:Skull Neoplasms / Q000601:surgery; D013100:Sphenoid Bone / Q000601:surgery