[Oral, facial and cranial manifestations of von Recklinghausen neurofibromatosis (NF)].
19 patients with neurofibromatosis (NF) type 1 were evaluated retrospectively. Many patients presented not only with orocraniofacial neurofibromas but also with cranial skeletal deformities--involving skullbase, skull, orbit, midface and mandibula--and ophthalmological and dental symptoms, as well as neurological disorders. Malignant transformation of NF was observed in 1 case. Severe manifestations of NF were associated with the plexiform growth type. Due to the nature of NF surgical treatment most often is only palliative.
Keutel
C
C
Klinik und Poliklinik für Mund-, Kiefer- und Gesichtschirurgie, Eberhard-Karls-Universität, Tübingen.
Vees
B
B
Krimmel
M
M
Cornelius
C P
CP
Schwenzer
N
N
D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D002675:Child, Preschool; D005145:Face / Q000473:pathology; D005153:Facial Neoplasms / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008875:Middle Aged; D009055:Mouth / Q000473:pathology; D009062:Mouth Neoplasms / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology; D009456:Neurofibromatosis 1 / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology; D012886:Skull / Q000473:pathology; D012888:Skull Neoplasms / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology